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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-08-12 19:09:59 UTC
Update Date2023-02-21 17:16:37 UTC
HMDB IDHMDB0003345
Secondary Accession Numbers
  • HMDB0061922
  • HMDB03345
  • HMDB61922
Metabolite Identification
Common Namealpha-D-Glucose
DescriptionAlpha-D-Glucose is the alpha anomer of D-Glucose. An anomer is an epimer at the hemiacetal/hemiketal carbon in a cyclic saccharide (such as glucose). Formally, glucose is a monosaccharide containing six carbon atoms and an aldehyde group. Therefore, it is referred to as an aldohexose. In solutions, glucose can exist in an open-chain (acyclic) and a ring (cyclic) form, the latter being the result of an intramolecular reaction between the aldehyde C atom and the C-5 hydroxyl group to form an intramolecular hemiacetal. In aqueous solution, both forms are in equilibrium and at pH 7 the cyclic one is predominant. Typically, the proportion of the linear form is <0.02%, the proportion of the alpha anomer (alpha-D-Glucose) is 36% and the proportion of the beta anomer (beta-D-Glucose) is 64%. The key difference between alpha and beta glucose is the orientation of hydroxyl (-OH) group attached to the first carbon atom. Different glucose anomers have different physical properties, melting points and specific rotations. For instance, alpha-D-glucose has a melting point of 146 oC, while beta-D-glucose has a melting point of 150 oC. When alpha-D-glucose molecules polymerize they form starch. When beta-D-glucose molecules polymerize, they form cellulose. Regardless of its anomeric form, glucose is a primary source of energy for all living organisms. It is a fundamental metabolite found in all organisms, ranging from bacteria to plants to humans. Most of the glucose generated on the Earth is made by plants and algae during photosynthesis from water and carbon dioxide, where it is used to make cellulose (and other polymeric forms of glucose called polysaccharides) that stabilize plant cell walls. Glucose is also found in fruits and other parts of plants in its free state. In animals, glucose can be generated from the breakdown of glycogen in a process known as glycogenolysis. Glucose can also be synthesized de novo in animals. In particular, it can be synthesized in the liver and kidneys from non-carbohydrate intermediates, such as pyruvate and glycerol (and gluconeogenic amino acids such as glycine, serine and alanine), by a process known as gluconeogenesis. Humans also consume large amounts of glucose as part of their regular diet. Ingested glucose initially binds to the receptor for sweet taste on the tongue in humans. This complex of the proteins T1R2 and T1R3 makes it possible to identify glucose-containing food sources. Glucose in the body mainly comes from food - about 300 g per day for the average adult. In humans, the breakdown of glucose-containing polysaccharides happens partly during chewing by means of the enzyme known as amylase, which is contained in saliva, as well as by other enzymes such as maltase, lactase and sucrase on the brush border of the small intestine. The blood sugar content of a healthy person in the short-time fasting state, e.g. after overnight fasting, is about 70 to 100 mg/dL of blood (4 to 5.5 mM). In blood plasma, the measured values are about 10-15% higher. Dysregulated metabolism of glucose can lead to a number of diseases including diabetes. Diabetes is a metabolic disorder where the body is unable to regulate levels of glucose in the blood either because of a lack of insulin in the body or the failure, by cells in the body, to respond properly to insulin. Each of these situations can be caused by persistently high elevations of blood glucose levels (called hyperglycemia), through pancreatic burnout and insulin resistance. Persistently elevated levels of glucose (>6 mM or >120 mg/dL) can lead to the formation of covalent adducts of glucose with plasma proteins through a non-enzymatic process known as glycation. This glycation reaction leads to advanced glycation end products or AGEs (PMID: 24634591 ). AGEs are thought to be the major causes of different diabetic complications. High glucose levels may induce glycation of various structural and functional proteins including plasma proteins and collagen. The non-enzymatic modification of plasma proteins such as albumin, fibrinogen, hemoglobin and globulins may produce various deleterious effects including alteration in drug binding in the plasma, platelet activation, generation of oxygen free radicals, impaired fibrinolysis and impairment in immune system regulation (PMID: 24634591 ). Transiently elevated glucose (up to 7.3 mM or 133 mg/dL) is often seen shortly after the consumption of a meal or a food item that is rich in carbohydrates -- even among very healthy people (PMID: 19885137 ). Glucose is also elevated when an individual is fighting viral or bacterial infections or suffering from traumatic injuries (burns, wounds). In fact, glucose can be significantly elevated (>11 mM or 200 mg/dL) when individuals are experiencing sepsis or septic shock (PMID: 16006275 ). On the other hand, low blood glucose levels (hypoglycemia) where blood glucose is <3.9 mM (70 mg/dL) are common among people with type 1 diabetes and people with type 2 diabetes who take certain diabetic medicines. Certain conditions, such as liver disease, may also cause low levels of blood glucose. Hypoglycemia can lead to fatigue, sleepiness, short temper or feeling faint.
Structure
Data?1676999797
Synonyms
ValueSource
alpha-D-GLCChEBI
alpha-DextroseChEBI
a-D-GLCGenerator
Α-D-GLCGenerator
a-DextroseGenerator
Α-dextroseGenerator
a-D-GlucoseGenerator
Α-D-glucoseGenerator
a-D-GlucopyranoseHMDB
a-GlucoseHMDB
alpha-D-GlucopyranoseHMDB
alpha-delta-GlucopyranoseHMDB
alpha-delta-GlucoseHMDB
alpha-GlucoseHMDB
HexopyranoseHMDB
Anhydrous dextroseHMDB
D GlucoseHMDB
D-GlucoseHMDB
DextroseHMDB
Dextrose, anhydrousHMDB
GlucoseHMDB
Glucose monohydrateHMDB
Glucose, (DL)-isomerHMDB
Glucose, (L)-isomerHMDB
Glucose, (alpha-D)-isomerHMDB
Glucose, (beta-D)-isomerHMDB
L GlucoseHMDB
L-GlucoseHMDB
Monohydrate, glucoseHMDB
1,3-alpha-D-GlucanHMDB
alpha-1,3-GlucanHMDB
alpha-D-GlucopyranosideHMDB
Chemical FormulaC6H12O6
Average Molecular Weight180.1559
Monoisotopic Molecular Weight180.063388116
IUPAC Name(2S,3R,4S,5S,6R)-6-(hydroxymethyl)oxane-2,3,4,5-tetrol
Traditional Nameα-glucose
CAS Registry Number492-62-6
SMILES
OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O
InChI Identifier
InChI=1S/C6H12O6/c7-1-2-3(8)4(9)5(10)6(11)12-2/h2-11H,1H2/t2-,3-,4+,5-,6+/m1/s1
InChI KeyWQZGKKKJIJFFOK-DVKNGEFBSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as hexoses. These are monosaccharides in which the sugar unit is a is a six-carbon containing moeity.
KingdomOrganic compounds
Super ClassOrganic oxygen compounds
ClassOrganooxygen compounds
Sub ClassCarbohydrates and carbohydrate conjugates
Direct ParentHexoses
Alternative Parents
Substituents
  • Hexose monosaccharide
  • Oxane
  • Secondary alcohol
  • Hemiacetal
  • Oxacycle
  • Organoheterocyclic compound
  • Polyol
  • Hydrocarbon derivative
  • Primary alcohol
  • Alcohol
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Naturally occurring process
RoleNot Available
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point146 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility500 mg/mL at 20 °CNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility782 g/LALOGPS
logP-2.6ALOGPS
logP-2.9ChemAxon
logS0.64ALOGPS
pKa (Strongest Acidic)11.3ChemAxon
pKa (Strongest Basic)-3ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area110.38 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity35.92 m³·mol⁻¹ChemAxon
Polarizability16.09 ųChemAxon
Number of Rings1ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+139.40131661259
DarkChem[M-H]-135.92631661259
DeepCCS[M+H]+141.38130932474
DeepCCS[M-H]-138.98530932474
DeepCCS[M-2H]-173.50930932474
DeepCCS[M+Na]+147.82730932474
AllCCS[M+H]+140.832859911
AllCCS[M+H-H2O]+136.732859911
AllCCS[M+NH4]+144.732859911
AllCCS[M+Na]+145.832859911
AllCCS[M-H]-131.832859911
AllCCS[M+Na-2H]-132.832859911
AllCCS[M+HCOO]-134.032859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
alpha-D-GlucoseOC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O3448.8Standard polar33892256
alpha-D-GlucoseOC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O1916.4Standard non polar33892256
alpha-D-GlucoseOC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O1649.9Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
alpha-D-Glucose,1TMS,isomer #1C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O1738.4Semi standard non polar33892256
alpha-D-Glucose,1TMS,isomer #2C[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O1673.8Semi standard non polar33892256
alpha-D-Glucose,1TMS,isomer #3C[Si](C)(C)O[C@H]1[C@@H](O)O[C@H](CO)[C@@H](O)[C@@H]1O1683.6Semi standard non polar33892256
alpha-D-Glucose,1TMS,isomer #4C[Si](C)(C)O[C@@H]1[C@@H](O)[C@@H](O)O[C@H](CO)[C@H]1O1688.3Semi standard non polar33892256
alpha-D-Glucose,1TMS,isomer #5C[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O)[C@H](O)[C@H]1O1678.0Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #1C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O)[C@@H](O)[C@@H]1O1722.4Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #10C[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O)[C@H](O)[C@H]1O[Si](C)(C)C1681.6Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #2C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C)[C@@H](O)[C@@H]1O1737.0Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #3C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H]1O1742.6Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #4C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O[Si](C)(C)C1712.2Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #5C[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O[Si](C)(C)C)[C@H](O)[C@H]1O1701.4Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #6C[Si](C)(C)O[C@H]1[C@H](O)[C@@H](CO)O[C@H](O[Si](C)(C)C)[C@@H]1O1703.5Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #7C[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O[Si](C)(C)C1679.6Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #8C[Si](C)(C)O[C@H]1[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H](O)O[C@@H]1CO1697.1Semi standard non polar33892256
alpha-D-Glucose,2TMS,isomer #9C[Si](C)(C)O[C@@H]1[C@@H](O[Si](C)(C)C)[C@@H](O)O[C@H](CO)[C@H]1O1692.2Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #1C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)[C@@H](O)[C@@H]1O1703.6Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #10C[Si](C)(C)O[C@@H]1[C@@H](O[Si](C)(C)C)[C@@H](O)O[C@H](CO)[C@H]1O[Si](C)(C)C1736.7Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #2C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H]1O1753.4Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #3C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O)[C@@H](O)[C@@H]1O[Si](C)(C)C1717.5Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #4C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C)[C@@H](O[Si](C)(C)C)[C@@H]1O1748.2Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #5C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C)[C@@H](O)[C@@H]1O[Si](C)(C)C1766.0Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #6C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1701.0Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #7C[Si](C)(C)O[C@H]1[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H](O[Si](C)(C)C)O[C@@H]1CO1733.4Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #8C[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)[C@H]1O1739.0Semi standard non polar33892256
alpha-D-Glucose,3TMS,isomer #9C[Si](C)(C)O[C@H]1[C@H](O)[C@@H](CO)O[C@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1725.4Semi standard non polar33892256
alpha-D-Glucose,4TMS,isomer #1C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)[C@@H](O[Si](C)(C)C)[C@@H]1O1815.5Semi standard non polar33892256
alpha-D-Glucose,4TMS,isomer #2C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)[C@@H](O)[C@@H]1O[Si](C)(C)C1815.2Semi standard non polar33892256
alpha-D-Glucose,4TMS,isomer #3C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O)[C@@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1820.5Semi standard non polar33892256
alpha-D-Glucose,4TMS,isomer #4C[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C)[C@@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1819.4Semi standard non polar33892256
alpha-D-Glucose,4TMS,isomer #5C[Si](C)(C)O[C@H]1[C@H](O[Si](C)(C)C)[C@@H](CO)O[C@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1800.9Semi standard non polar33892256
alpha-D-Glucose,5TMS,isomer #1C[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)[C@@H](O[Si](C)(C)C)[C@@H]1O[Si](C)(C)C1877.4Semi standard non polar33892256
alpha-D-Glucose,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O2002.6Semi standard non polar33892256
alpha-D-Glucose,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O1940.1Semi standard non polar33892256
alpha-D-Glucose,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)O[C@H]1[C@@H](O)O[C@H](CO)[C@@H](O)[C@@H]1O1940.5Semi standard non polar33892256
alpha-D-Glucose,1TBDMS,isomer #4CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](O)[C@@H](O)O[C@H](CO)[C@H]1O1947.1Semi standard non polar33892256
alpha-D-Glucose,1TBDMS,isomer #5CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O)[C@H](O)[C@H]1O1943.8Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)[C@@H](O)[C@@H]1O2208.7Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #10CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O)[C@H](O)[C@H]1O[Si](C)(C)C(C)(C)C2181.6Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)[C@@H]1O2210.9Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O2219.8Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O)[C@@H]1O[Si](C)(C)C(C)(C)C2197.5Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #5CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](CO)O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)[C@H]1O2193.9Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #6CC(C)(C)[Si](C)(C)O[C@H]1[C@H](O)[C@@H](CO)O[C@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O2195.0Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #7CC(C)(C)[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O[Si](C)(C)C(C)(C)C2180.7Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #8CC(C)(C)[Si](C)(C)O[C@H]1[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)O[C@@H]1CO2190.8Semi standard non polar33892256
alpha-D-Glucose,2TBDMS,isomer #9CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)O[C@H](CO)[C@H]1O2192.8Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)[C@@H]1O2441.4Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #10CC(C)(C)[Si](C)(C)O[C@@H]1[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)O[C@H](CO)[C@H]1O[Si](C)(C)C(C)(C)C2455.2Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O2449.6Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)[C@@H](O)[C@@H]1O[Si](C)(C)C(C)(C)C2445.1Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #4CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O2466.7Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #5CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)[C@@H]1O[Si](C)(C)C(C)(C)C2468.7Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #6CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2439.7Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #7CC(C)(C)[Si](C)(C)O[C@H]1[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H](O[Si](C)(C)C(C)(C)C)O[C@@H]1CO2451.3Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #8CC(C)(C)[Si](C)(C)O[C@H]1O[C@H](CO)[C@@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)[C@H]1O2451.2Semi standard non polar33892256
alpha-D-Glucose,3TBDMS,isomer #9CC(C)(C)[Si](C)(C)O[C@H]1[C@H](O)[C@@H](CO)O[C@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2454.9Semi standard non polar33892256
alpha-D-Glucose,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O2671.4Semi standard non polar33892256
alpha-D-Glucose,4TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O)[C@@H]1O[Si](C)(C)C(C)(C)C2668.4Semi standard non polar33892256
alpha-D-Glucose,4TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2664.4Semi standard non polar33892256
alpha-D-Glucose,4TBDMS,isomer #4CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2681.0Semi standard non polar33892256
alpha-D-Glucose,4TBDMS,isomer #5CC(C)(C)[Si](C)(C)O[C@H]1[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](CO)O[C@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2669.4Semi standard non polar33892256
alpha-D-Glucose,5TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC[C@H]1O[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)[C@@H](O[Si](C)(C)C(C)(C)C)[C@@H]1O[Si](C)(C)C(C)(C)C2890.3Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - alpha-D-Glucose GC-MS (Non-derivatized) - 70eV, Positivesplash10-0np0-9700000000-e8d638dc817e46b97d7b2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-D-Glucose GC-MS (5 TMS) - 70eV, Positivesplash10-004i-6122690000-eaf6f7adf34ccd0c667b2017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-D-Glucose GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 10V, Negative-QTOFsplash10-0a4i-9100000000-57ee067515dbaeec1e272021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 35V, Negative-QTOFsplash10-0ab9-9100000000-311a61760b7c0dc3a7e12021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 35V, Negative-QTOFsplash10-0ab9-9100000000-f2beec08615d57ac1a022021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 35V, Negative-QTOFsplash10-0ab9-9100000000-eb694004566014e0b2672021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 20V, Negative-QTOFsplash10-0a4i-9000000000-906b2fd231873b000e082021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose 40V, Negative-QTOFsplash10-0a4i-9000000000-3cd19dc7a445df26b86d2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-000i-9100000000-53278ff57ca00e1b5d1a2012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-D-Glucose Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0079-9000000000-c4182c64988208ac78b02012-07-25HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 10V, Negative-QTOFsplash10-004i-2900000000-a4ec4f0b1e29e360a9522016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 20V, Negative-QTOFsplash10-01t9-6900000000-7b3ea9c64ecc8d4ac8672016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 40V, Negative-QTOFsplash10-052f-9100000000-ec2bf4918640a0a363982016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 10V, Negative-QTOFsplash10-004i-2900000000-2448926b508622464fe72021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 20V, Negative-QTOFsplash10-056r-8900000000-4c073cb93b78120113e62021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 40V, Negative-QTOFsplash10-0a4l-9000000000-ece70093ab5d3c331ac42021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 10V, Positive-QTOFsplash10-01q9-0900000000-b0bc47623e7b2ca31c022016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 20V, Positive-QTOFsplash10-03ea-3900000000-648e1637af29cf2a35182016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 40V, Positive-QTOFsplash10-0007-9200000000-9e6f46a1cbf52d6e347a2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 10V, Positive-QTOFsplash10-01qa-0900000000-04ceb34d441ff6a757632021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 20V, Positive-QTOFsplash10-03dl-9400000000-a8f1ceab155611f949c12021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-D-Glucose 40V, Positive-QTOFsplash10-01ow-9000000000-358f68fc2b7a72c275462021-09-25Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
  • Extracellular
  • Lysosome
  • Endoplasmic reticulum
  • Golgi apparatus
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Adipose Tissue
  • Adrenal Cortex
  • Adrenal Gland
  • Adrenal Medulla
  • Bladder
  • Brain
  • Epidermis
  • Eye Lens
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Lung
  • Neuron
  • Ovary
  • Pancreas
  • Placenta
  • Prostate
  • Skeletal Muscle
  • Testis
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified4000.0-6000.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified2200.0 (1100.0 - 3300.0) uMNewborn (0-30 days old)BothNormal
    • Wu AHB (2006) Tie...
details
BloodDetected and Quantified2750.0 (2200.0-3300.0) uMNewborn (0-30 days old)BothNormal
    • Wu AHB (2006) Tie...
details
BloodDetected and Quantified4450.0 (3300.0 - 5600.0) uMChildren (1-13 years old)BothNormal
    • Wu AHB (2006) Tie...
details
BloodDetected and Quantified5450.0 (4200.0 - 6700.0) uMElderly (>65 years old)BothNormal
    • Wu AHB (2006) Tie...
details
BloodDetected and Quantified4750.0 +/- 184.0 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified4440.0 +/- 370.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified4570.0 +/- 411.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified4470.0 +/- 346.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified4380 +/-275 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified<5994.808 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified5000 +/- 600 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified4500 +/- 1000 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified5300.0 (4900.0 - 5700.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified4163.0610-6383.360 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified3100-5600 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified5400.0 (4700.0 - 6100.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified2800-5000 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified3500-7000 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified5181.0 +/- 394.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified5350.0 +/- 120.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified4440.0 +/- 100.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified4470.0 +/- 110.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified4971.3 +/- 372.8 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified3300-5600 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified3885.524-6105.823 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified4894.19(1723.06) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified4823.600 +/- 249.784 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified4695.933 +/- 327.494 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified3300-6900 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified3885.524-6105.823 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified3500-6000 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified4200-6300 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified4860 (4670-5190) uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified3890-5550 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified4500.0+/- 100.0 uMAdult (>18 years old)FemaleNormal details
Breast MilkDetected and Quantified1500 +/- 530 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified5390.0 +/- 1650.0 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2775.374-3885.524 uMInfant (0-1 year old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1720.0 (1560.0-1880.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2960 +/- 1110 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2500-4000 uMChildren (1-13 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified743 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified18271 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified9717 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified1-2762 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified4-389 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified20 (<1-914) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified197 (<1-10764) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified247 (<1-6510) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified90 (<1-1994) uMAdult (>18 years old)Female
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified355.05 +/- 260.74 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified817.300 +/- 1070.700 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified230-300 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected and Quantified143.14 +/- 399.78 umol/mmol creatinineInfant (0-1 year old)FemaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
UrineDetected and Quantified35.6 (10.3-56.7) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified37.5 (12.5-58.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified76-800 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified106.3-1193 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified507.9 +/- 275.0 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified421.0 +/- 219.7 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified7.0 (0.0-15.0) umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified25.8 +/- 13.82 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified15.0 (0.0-50.0) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified9.0 (0.0-19.0) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified31.12 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified16.66-111.07 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam. ...
details
UrineDetected and Quantified31.598 +/- 15.976 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified4400.0 (3300.0-5500.0) uMAdult (>18 years old)Both3-methyl-crotonyl-glycinuria
    • MetaGene: Metabol...
details
BloodDetected and Quantified1750.0 (500.0-3000.0) uMChildren (1-13 years old)Both3-Methyl-crotonyl-glycinuria
    • MetaGene: Metabol...
details
BloodDetected and Quantified1750.00 (500.00-3000.00) uMChildren (1-13 years old)BothHypoadrenocorticism
    • MetaGene: Metabol...
details
BloodDetected and Quantified>11100 uMAdult (>18 years old)Both
Diabetes mellitus
    • World Health Orga...
details
BloodDetected and Quantified>7000 uMAdult (>18 years old)Both
Diabetes mellitus
    • World Health Orga...
details
BloodDetected and Quantified2164.792 uMChildren (1-13 years old)Female
Fanconi Bickel syndrome
    • Late Diagnosis of...
details
BloodDetected and Quantified42460 uMInfant (0-1 year old)FemaleWolcott-Rallison syndrome
    • Neonatal diabetes...
details
BloodDetected and Quantified1909.457 +/- 233.131 uMInfant (0-1 year old)Male
Glucagon deficiency
details
BloodDetected and Quantified<2600 uMNewborn (0-30 days old)BothBeckwith-Wiedemann Syndrome details
BloodDetected and Quantified900-2200 uMInfant (0-1 year old)Both
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
details
BloodDetected and Quantified200-2200 uMNewborn (0-30 days old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
BloodDetected and Quantified600-1000 uMNewborn (0-30 days old)BothHyperinsulinemic hypoglycemia, familial, 1, HHF1 details
BloodDetected and Quantified390 uMInfant (0-1 year old)FemaleLong-chain Fatty Acids, Defect in Transport of details
BloodDetected and Quantified390 uMInfant (0-1 year old)FemaleCarnitine transporter defect; primary systemic carnitine deficiency details
BloodDetected and Quantified444.0598-555.0748 uMChildren (1-13 years old)FemaleFructose-1,6-bisphosphatase deficiency details
BloodDetected and Quantified1110.15 uMInfant (0-1 year old)Female
Fructose-1,6-bisphosphatase deficiency
details
BloodDetected and Quantified2220.299 uMInfant (0-1 year old)Female
Fructose-1,6-bisphosphatase deficiency
details
BloodDetected and Quantified2553.344 uMInfant (0-1 year old)MaleLeigh Syndrome details
BloodDetected and Quantified2775.374-10213.376 uMAdult (>18 years old)FemaleProprotein Convertase 1/3 Deficiency details
BloodDetected and Quantified9300 +/- 5000 uMAdult (>18 years old)Both
Diabetes and Deafness, Maternally Inherited
details
BloodDetected and Quantified6300 +/- 2000 uMAdult (>18 years old)BothDiabetes and Deafness, Maternally Inherited details
BloodDetected and Quantified2800 uMChildren (1-13 years old)Male
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
details
BloodDetected and Quantified1000-4100 uMChildren (1-13 years old)Male3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency details
BloodDetected and Quantified1940 +/- 570 uMChildren (1-13 years old)Both
Hyperinsulinemic hypoglycemia, familial, 1, HHF1
details
BloodDetected and Quantified1900 +/- 700 uMChildren (1-13 years old)Both
Hyperinsulinemic hypoglycemia, familial, 1, HHF1
details
BloodDetected and Quantified4995.673-5106.688 uMAdult (>18 years old)Male
Primary Hypomagnesemia
details
BloodDetected and Quantified4329.583 uMInfant (0-1 year old)FemaleLeptin Deficiency or Dysfunction details
BloodDetected and Quantified2800 uMNewborn (0-30 days old)Male
2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD)
details
BloodDetected and Quantified5828.285-15264.557 uMAdult (>18 years old)Female
21-hydroxylase deficiency
details
BloodDetected and Quantified2997.404 uMAdolescent (13-18 years old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified3108.419 uMAdult (>18 years old)Male
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified2053.777-2220.299 uMChildren (1-13 years old)Both
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified2164.792-2553.344 uMInfant (0-1 year old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified1500 uMChildren (1-13 years old)MaleCarnitine palmitoyltransferase deficiency I details
BloodDetected and Quantified<100 uMNewborn (0-30 days old)MaleCarnitine palmitoyltransferase deficiency I details
BloodDetected and Quantified6000.0 (5000.0 - 6800.0) uMAdult (>18 years old)BothAcute myelogenous leukemia (AML) details
BloodDetected and Quantified1300-1400 uMInfant (0-1 year old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified<2600 uMInfant (0-1 year old)Not Specified
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
details
BloodDetected and Quantified2300 uMInfant (0-1 year old)Female
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
details
BloodDetected and Quantified700-2600 uMInfant (0-1 year old)Not SpecifiedMitochondrial trifunctional protein deficiency details
BloodDetected and Quantified1665.224-2775.374 uMInfant (0-1 year old)Female
Hypoglycemia, familial neonatal
details
BloodDetected and Quantified800-1600 uMNewborn (0-30 days old)Both
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified5114.0 +/- 483.0 uMAdult (>18 years old)BothHyperlipidaemia details
BloodDetected and Quantified2200 uMNewborn (0-30 days old)Male
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency
details
BloodDetected and Quantified5500.0 +/- 160.0 uMAdult (>18 years old)Both
Growth hormone deficiency
details
BloodDetected and Quantified333.0449 uMNewborn (0-30 days old)Female
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
details
BloodDetected and Quantified555.0748 uMNewborn (0-30 days old)Female
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
details
BloodDetected and Quantified5600-7600 uMAdult (>18 years old)Both
Maturity onset diabetes of the young, type 2
details
BloodDetected and Quantified3743 +/- 1272.9 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified4194.1 (1229.6) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified3702.2 (713.5) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified4312.9 (1783.0) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified3362.4 (765.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified245.5 (96.0) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified217.3 (73.8) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified2100-2700 uMInfant (0-1 year old)Male3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
BloodDetected and Quantified4133.0 +/- 903.7 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified3848.99 +/- 783.5 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified166.522 uMNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
BloodDetected and Quantified3241.19 (898.95) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified3171.93 (754.22) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified5535.11(1872.49) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified4812.499 +/- 288.639 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified5112.239 +/- 427.408 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified4235.221 +/- 55.507 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified4390.642 +/- 316.393 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified400 uMInfant (0-1 year old)FemaleInfantile Liver Failure Syndrome 2 details
BloodDetected and Quantified500-5200 uMChildren (1-13 years old)BothInfantile Liver Failure Syndrome 2 details
BloodDetected and Quantified1665.224 uMChildren (1-13 years old)FemaleMetabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration details
BloodDetected and Quantified943.627 uMInfant (0-1 year old)FemaleMetabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration details
BloodDetected and Quantified1332.180-9436.272 uMNewborn (0-30 days old)MaleDonohue Syndrome details
BloodDetected and Quantified2800 uMChildren (1-13 years old)MalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
BloodDetected and Quantified7800(6500-12000) uMAdult (>18 years old)BothSepsis details
BloodDetected and Quantified900 uMNewborn (0-30 days old)FemalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
BloodDetected and Quantified800-3200 uMChildren (1-13 years old)BothPhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
BloodDetected and Quantified6150 (5000-10090) uMAdult (>18 years old)BothLipodystrophy details
BloodDetected and Quantified7830 (5220-10890) uMAdult (>18 years old)BothLipodystrophy, Congenital Generalized details
BloodDetected and Quantified5060 (4670-6560) uMAdult (>18 years old)BothPartial lipodystrophy details
BloodDetected and Quantified6810 (5210-10250) uMAdult (>18 years old)BothFamilial partial lipodystrophy details
BloodDetected and Quantified3890 uMInfant (0-1 year old)Male3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified200 uMInfant (0-1 year old)FemaleLong-chain Fatty Acids, Defect in Transport of details
Cerebrospinal Fluid (CSF)Detected and Quantified200 uMInfant (0-1 year old)FemaleCarnitine transporter defect; primary systemic carnitine deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified2053.777 uMInfant (0-1 year old)MaleLeigh Syndrome details
Cerebrospinal Fluid (CSF)Detected and Quantified3700.0 (3200.0-4200.0) uMAdult (>18 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified1837.260 +/- 271.980 uMChildren (1-13 years old)BothGlucose transporter type 1 deficiency syndrome details
Cerebrospinal Fluid (CSF)Detected and Quantified1900 uMChildren (1-13 years old)FemaleGLUT1 deficiency syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleGout details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femaleankylosing spondylitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femalerheumatoid arthritis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable Bowel Syndrome
details
FecesDetected but not QuantifiedNot QuantifiedNewborn (0-30 days old)Not Specified
Premature neonates
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothImmunoglobulin A nephropathy (IgAN) progressor details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specifiedasymptomatic diverticulosis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specifiedsymptomatic uncomplicated diverticular disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis
details
UrineDetected and Quantified19700 umol/mmol creatinineAdult (>18 years old)BothDiabetes details
UrineDetected and Quantified79600.0 (68300.0-90900.0) umol/mmol creatinineAdult (>18 years old)Both
Diabetes
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified17449.11 (137.28 - 129930.33) umol/mmol creatinineAdult (>18 years old)BothType 1 diabetes Mellitus
    • Lorena Ivona ŞTEF...
details
UrineDetected and Quantified30.015 +/- 27.82 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified>5550.748 umol/mmol creatinineChildren (1-13 years old)FemaleFanconi Bickel syndrome
    • Late Diagnosis of...
details
Associated Disorders and Diseases
Disease References
Glucagon deficiency
  1. Vidnes J, Oyasaeter S: Glucagon deficiency causing severe neonatal hypoglycemia in a patient with normal insulin secretion. Pediatr Res. 1977 Sep;11(9 Pt 1):943-9. [PubMed:904979 ]
Beckwith-Wiedemann Syndrome
  1. Martinez y Martinez R, Martinez-Carboney R, Ocampo-Campos R, Rivera H, Gomez Plascencia y Castillo J, Cuevas A, Martin Manrique MC: Wiedemann-Beckwith syndrome: clinical, cytogenetical and radiological observations in 39 new cases. Genet Couns. 1992;3(2):67-76. [PubMed:1642813 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Ozand PT, al Aqeel A, Gascon G, Brismar J, Thomas E, Gleispach H: 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency in Saudi Arabia. J Inherit Metab Dis. 1991;14(2):174-88. [PubMed:1886403 ]
  2. Zschocke J, Penzien JM, Bielen R, Casals N, Aledo R, Pie J, Hoffmann GF, Hegardt FG, Mayatepek E: The diagnosis of mitochondrial HMG-CoA synthase deficiency. J Pediatr. 2002 Jun;140(6):778-80. doi: 10.1067/mpd.2002.123854. [PubMed:12072887 ]
  3. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
Hyperinsulinemic hypoglycemia, familial, 1, HHF1
  1. Horev Z, Ipp M, Levey P, Daneman D: Familial hyperinsulinism: successful conservative management. J Pediatr. 1991 Nov;119(5):717-20. [PubMed:1941376 ]
  2. Touati G, Poggi-Travert F, Ogier de Baulny H, Rahier J, Brunelle F, Nihoul-Fekete C, Czernichow P, Saudubray JM: Long-term treatment of persistent hyperinsulinaemic hypoglycaemia of infancy with diazoxide: a retrospective review of 77 cases and analysis of efficacy-predicting criteria. Eur J Pediatr. 1998 Aug;157(8):628-33. [PubMed:9727845 ]
Long-chain Fatty Acids, Defect in Transport of
  1. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med. 1988 Nov 17;319(20):1331-6. doi: 10.1056/NEJM198811173192006. [PubMed:3185635 ]
Carnitine transporter defect; primary systemic carnitine deficiency
  1. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med. 1988 Nov 17;319(20):1331-6. doi: 10.1056/NEJM198811173192006. [PubMed:3185635 ]
Fructose-1,6-diphosphatase deficiency
  1. Baker L, Winegrad AI: Fasting hypoglycaemia and metabolic acidosis associated with deficiency of hepatic fructose-1,6-diphosphatase activity. Lancet. 1970 Jul 4;2(7662):13-6. [PubMed:4193749 ]
  2. Pagliara AS, Karl IE, Keating JP, Brown BI, Kipnis DM: Hepatic fructose-1,6-diphosphatase deficiency. A cause of lactic acidosis and hypoglycemia in infancy. J Clin Invest. 1972 Aug;51(8):2115-23. doi: 10.1172/JCI107018. [PubMed:4341015 ]
Leigh's syndrome, subacute necrotizing encephalopathy, SNE
  1. Hommes FA, Polman HA, Reerink JD: Leigh's encephalomyelopathy: an inborn error of gluconeogenesis. Arch Dis Child. 1968 Aug;43(230):423-6. [PubMed:4873809 ]
Proprotein Convertase 1/3 Deficiency
  1. O'Rahilly S, Gray H, Humphreys PJ, Krook A, Polonsky KS, White A, Gibson S, Taylor K, Carr C: Brief report: impaired processing of prohormones associated with abnormalities of glucose homeostasis and adrenal function. N Engl J Med. 1995 Nov 23;333(21):1386-90. doi: 10.1056/NEJM199511233332104. [PubMed:7477119 ]
Diabetes and Deafness, Maternally Inherited
  1. Velho G, Byrne MM, Clement K, Sturis J, Pueyo ME, Blanche H, Vionnet N, Fiet J, Passa P, Robert JJ, Polonsky KS, Froguel P: Clinical phenotypes, insulin secretion, and insulin sensitivity in kindreds with maternally inherited diabetes and deafness due to mitochondrial tRNALeu(UUR) gene mutation. Diabetes. 1996 Apr;45(4):478-87. [PubMed:8603770 ]
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Thompson GN, Hsu BY, Pitt JJ, Treacy E, Stanley CA: Fasting hypoketotic coma in a child with deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase. N Engl J Med. 1997 Oct 23;337(17):1203-7. doi: 10.1056/NEJM199710233371704. [PubMed:9337379 ]
  2. Morris AA, Lascelles CV, Olpin SE, Lake BD, Leonard JV, Quant PA: Hepatic mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase deficiency. Pediatr Res. 1998 Sep;44(3):392-6. doi: 10.1203/00006450-199809000-00021. [PubMed:9727719 ]
  3. Conboy E, Vairo F, Schultz M, Agre K, Ridsdale R, Deyle D, Oglesbee D, Gavrilov D, Klee EW, Lanpher B: Mitochondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency: Unique Presenting Laboratory Values and a Review of Biochemical and Clinical Features. JIMD Rep. 2017 Oct 14. doi: 10.1007/8904_2017_59. [PubMed:29030856 ]
Primary hypomagnesemia
  1. Jin-no Y, Kamiya Y, Okada M, Hirako M, Takada N, Kawaguchi M: Primary hypomagnesemia caused by isolated magnesium malabsorption: atypical case in adult. Intern Med. 1999 Mar;38(3):261-5. [PubMed:10337938 ]
Leptin Deficiency or Dysfunction
  1. Farooqi IS, Jebb SA, Langmack G, Lawrence E, Cheetham CH, Prentice AM, Hughes IA, McCamish MA, O'Rahilly S: Effects of recombinant leptin therapy in a child with congenital leptin deficiency. N Engl J Med. 1999 Sep 16;341(12):879-84. doi: 10.1056/NEJM199909163411204. [PubMed:10486419 ]
21-Hydroxylase deficiency
  1. Warinner SA, Zimmerman D, Thompson GB, Grant CS: Study of three patients with congenital adrenal hyperplasia treated by bilateral adrenalectomy. World J Surg. 2000 Nov;24(11):1347-52. [PubMed:11038205 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA: Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr. 2001 Mar;138(3):383-9. doi: 10.1067/mpd.2001.111818. [PubMed:11241047 ]
  2. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
  3. Molven A, Matre GE, Duran M, Wanders RJ, Rishaug U, Njolstad PR, Jellum E, Sovik O: Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation. Diabetes. 2004 Jan;53(1):221-7. [PubMed:14693719 ]
  4. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
Carnitine palmitoyltransferase I deficiency
  1. Olpin SE, Allen J, Bonham JR, Clark S, Clayton PT, Calvin J, Downing M, Ives K, Jones S, Manning NJ, Pollitt RJ, Standing SJ, Tanner MS: Features of carnitine palmitoyltransferase type I deficiency. J Inherit Metab Dis. 2001 Feb;24(1):35-42. [PubMed:11286380 ]
Acute myelogenous leukemia
  1. Tatidis L, Vitols S, Gruber A, Paul C, Axelson M: Cholesterol catabolism in patients with acute myelogenous leukemia and hypocholesterolemia: suppressed levels of a circulating marker for bile acid synthesis. Cancer Lett. 2001 Sep 20;170(2):169-75. [PubMed:11463495 ]
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Mitochondrial trifunctional protein deficiency
  1. den Boer ME, Dionisi-Vici C, Chakrapani A, van Thuijl AO, Wanders RJ, Wijburg FA: Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement. J Pediatr. 2003 Jun;142(6):684-9. doi: 10.1067/mpd.2003.231. [PubMed:12838198 ]
Hypoglycemia, familial neonatal
  1. COCHRANE WA, PAYNE WW, SIMPKISS MJ, WOOLF LI: Familial hypoglycemia precipitated by amino acids. J Clin Invest. 1956 Apr;35(4):411-22. doi: 10.1172/JCI103292. [PubMed:13306783 ]
Hyperlipoproteinemia
  1. Sinha S, Misra A, Kumar V, Jagannathan NR, Bal CS, Pandey RM, Singhania R, Deepak: Proton magnetic resonance spectroscopy and single photon emission computed tomography study of the brain in asymptomatic young hyperlipidaemic Asian Indians in North India show early abnormalities. Clin Endocrinol (Oxf). 2004 Aug;61(2):182-9. [PubMed:15272912 ]
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency
  1. Perez-Cerda C, Garcia-Villoria J, Ofman R, Sala PR, Merinero B, Ramos J, Garcia-Silva MT, Beseler B, Dalmau J, Wanders RJ, Ugarte M, Ribes A: 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: an X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease. Pediatr Res. 2005 Sep;58(3):488-91. [PubMed:16148061 ]
Growth hormone deficiency
  1. Darzy KH, Murray RD, Gleeson HK, Pezzoli SS, Thorner MO, Shalet SM: The impact of short-term fasting on the dynamics of 24-hour growth hormone (GH) secretion in patients with severe radiation-induced GH deficiency. J Clin Endocrinol Metab. 2006 Mar;91(3):987-94. Epub 2005 Dec 29. [PubMed:16384844 ]
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
  1. Kim CJ, Lin L, Huang N, Quigley CA, AvRuskin TW, Achermann JC, Miller WL: Severe combined adrenal and gonadal deficiency caused by novel mutations in the cholesterol side chain cleavage enzyme, P450scc. J Clin Endocrinol Metab. 2008 Mar;93(3):696-702. doi: 10.1210/jc.2007-2330. Epub 2008 Jan 8. [PubMed:18182448 ]
Maturity onset diabetes of the young, type 2
  1. Estalella I, Garcia-Gimeno MA, Marina A, Castano L, Sanz P: Biochemical characterization of novel glucokinase mutations isolated from Spanish maturity-onset diabetes of the young (MODY2) patients. J Hum Genet. 2008;53(5):460-6. doi: 10.1007/s10038-008-0271-5. Epub 2008 Mar 6. [PubMed:18322640 ]
Schizophrenia
  1. Xuan J, Pan G, Qiu Y, Yang L, Su M, Liu Y, Chen J, Feng G, Fang Y, Jia W, Xing Q, He L: Metabolomic profiling to identify potential serum biomarkers for schizophrenia and risperidone action. J Proteome Res. 2011 Dec 2;10(12):5433-43. doi: 10.1021/pr2006796. Epub 2011 Nov 8. [PubMed:22007635 ]
  2. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
  3. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
  2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
  3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
  4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
  5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
  1. Benkert AR, Young M, Robinson D, Hendrickson C, Lee PA, Strauss KA: Severe Salt-Losing 3beta-Hydroxysteroid Dehydrogenase Deficiency: Treatment and Outcomes of HSD3B2 c.35G>A Homozygotes. J Clin Endocrinol Metab. 2015 Aug;100(8):E1105-15. doi: 10.1210/jc.2015-2098. Epub 2015 Jun 16. [PubMed:26079780 ]
Infantile Liver Failure Syndrome 2
  1. Staufner C, Haack TB, Kopke MG, Straub BK, Kolker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Lenz D, Kuhr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Ozolek JA, Zschocke J, Kuster A, Dick A, Das AM, Wieland T, Terrile C, Strom TM, Meitinger T, Prokisch H, Hoffmann GF: Recurrent acute liver failure due to NBAS deficiency: phenotypic spectrum, disease mechanisms, and therapeutic concepts. J Inherit Metab Dis. 2016 Jan;39(1):3-16. doi: 10.1007/s10545-015-9896-7. Epub 2015 Nov 5. [PubMed:26541327 ]
Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration
  1. Lalani SR, Liu P, Rosenfeld JA, Watkin LB, Chiang T, Leduc MS, Zhu W, Ding Y, Pan S, Vetrini F, Miyake CY, Shinawi M, Gambin T, Eldomery MK, Akdemir ZH, Emrick L, Wilnai Y, Schelley S, Koenig MK, Memon N, Farach LS, Coe BP, Azamian M, Hernandez P, Zapata G, Jhangiani SN, Muzny DM, Lotze T, Clark G, Wilfong A, Northrup H, Adesina A, Bacino CA, Scaglia F, Bonnen PE, Crosson J, Duis J, Maegawa GH, Coman D, Inwood A, McGill J, Boerwinkle E, Graham B, Beaudet A, Eng CM, Hanchard NA, Xia F, Orange JS, Gibbs RA, Lupski JR, Yang Y: Recurrent Muscle Weakness with Rhabdomyolysis, Metabolic Crises, and Cardiac Arrhythmia Due to Bi-allelic TANGO2 Mutations. Am J Hum Genet. 2016 Feb 4;98(2):347-57. doi: 10.1016/j.ajhg.2015.12.008. Epub 2016 Jan 21. [PubMed:26805781 ]
Donohue Syndrome
  1. Nijim Y, Awni Y, Adawi A, Bowirrat A: Classic Case Report of Donohue Syndrome (Leprechaunism; OMIM *246200): The Impact of Consanguineous Mating. Medicine (Baltimore). 2016 Feb;95(6):e2710. doi: 10.1097/MD.0000000000002710. [PubMed:26871809 ]
Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic
  1. Santra S, Cameron JM, Shyr C, Zhang L, Drogemoller B, Ross CJ, Wasserman WW, Wevers RA, Rodenburg RJ, Gupte G, Preece MA, van Karnebeek CD: Cytosolic phosphoenolpyruvate carboxykinase deficiency presenting with acute liver failure following gastroenteritis. Mol Genet Metab. 2016 May;118(1):21-7. doi: 10.1016/j.ymgme.2016.03.001. Epub 2016 Mar 4. [PubMed:26971250 ]
  2. Vieira P, Cameron J, Rahikkala E, Keski-Filppula R, Zhang LH, Santra S, Matthews A, Myllynen P, Nuutinen M, Moilanen JS, Rodenburg RJ, Rolfs A, Uusimaa J, van Karnebeek CDM: Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction. Mol Genet Metab. 2017 Apr;120(4):337-341. doi: 10.1016/j.ymgme.2017.02.003. Epub 2017 Feb 6. [PubMed:28216384 ]
Sepsis
  1. Wernly B, Lichtenauer M, Hoppe UC, Jung C: Hyperglycemia in septic patients: an essential stress survival response in all, a robust marker for risk stratification in some, to be messed with in none. J Thorac Dis. 2016 Jul;8(7):E621-4. doi: 10.21037/jtd.2016.05.24. [PubMed:27501420 ]
Lipodystrophy
  1. Akinci G, Topaloglu H, Demir T, Danyeli AE, Talim B, Keskin FE, Kadioglu P, Talip E, Altay C, Yaylali GF, Bilen H, Nur B, Demir L, Onay H, Akinci B: Clinical spectra of neuromuscular manifestations in patients with lipodystrophy: A multicenter study. Neuromuscul Disord. 2017 Oct;27(10):923-930. doi: 10.1016/j.nmd.2017.05.015. Epub 2017 Jun 1. [PubMed:28754454 ]
Lipodystrophy, Congenital Generalized
  1. Akinci G, Topaloglu H, Demir T, Danyeli AE, Talim B, Keskin FE, Kadioglu P, Talip E, Altay C, Yaylali GF, Bilen H, Nur B, Demir L, Onay H, Akinci B: Clinical spectra of neuromuscular manifestations in patients with lipodystrophy: A multicenter study. Neuromuscul Disord. 2017 Oct;27(10):923-930. doi: 10.1016/j.nmd.2017.05.015. Epub 2017 Jun 1. [PubMed:28754454 ]
Partial lipodystrophy
  1. Akinci G, Topaloglu H, Demir T, Danyeli AE, Talim B, Keskin FE, Kadioglu P, Talip E, Altay C, Yaylali GF, Bilen H, Nur B, Demir L, Onay H, Akinci B: Clinical spectra of neuromuscular manifestations in patients with lipodystrophy: A multicenter study. Neuromuscul Disord. 2017 Oct;27(10):923-930. doi: 10.1016/j.nmd.2017.05.015. Epub 2017 Jun 1. [PubMed:28754454 ]
Familial partial lipodystrophy
  1. Akinci G, Topaloglu H, Demir T, Danyeli AE, Talim B, Keskin FE, Kadioglu P, Talip E, Altay C, Yaylali GF, Bilen H, Nur B, Demir L, Onay H, Akinci B: Clinical spectra of neuromuscular manifestations in patients with lipodystrophy: A multicenter study. Neuromuscul Disord. 2017 Oct;27(10):923-930. doi: 10.1016/j.nmd.2017.05.015. Epub 2017 Jun 1. [PubMed:28754454 ]
3-Methyl-crotonyl-glycinuria
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Addison's Disease
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Fanconi Bickel syndrome
  1. Nirupama Gupta, Bimota Nambam, David A. Weinstein, and Lawrence R. Shoemaker (2016). Late Diagnosis of Fanconi-Bickel Syndrome: Challenges With the Diagnosis and Literature Review . Journal of Inborn Errors of Metabolism and Screening.
Wolcott-Rallison syndrome
  1. Can Thi Bich Ngoc, Vu Chi Dung, Sarah Flanagan and Sian Ellard (2013). Neonatal diabetes in Wolcott-Rallison syndrome: a case report. International Journal of Pediatric Endocrinology.
Alzheimer's disease
  1. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed:9693263 ]
Glucose transporter type 1 deficiency syndrome
  1. Wang D, Pascual JM, Yang H, Engelstad K, Jhung S, Sun RP, De Vivo DC: Glut-1 deficiency syndrome: clinical, genetic, and therapeutic aspects. Ann Neurol. 2005 Jan;57(1):111-8. [PubMed:15622525 ]
  2. Koy A, Assmann B, Klepper J, Mayatepek E: Glucose transporter type 1 deficiency syndrome with carbohydrate-responsive symptoms but without epilepsy. Dev Med Child Neurol. 2011 Dec;53(12):1154-6. doi: 10.1111/j.1469-8749.2011.04082.x. Epub 2011 Aug 12. [PubMed:21838819 ]
Irritable bowel syndrome
  1. Ponnusamy K, Choi JN, Kim J, Lee SY, Lee CH: Microbial community and metabolomic comparison of irritable bowel syndrome faeces. J Med Microbiol. 2011 Jun;60(Pt 6):817-27. doi: 10.1099/jmm.0.028126-0. Epub 2011 Feb 17. [PubMed:21330412 ]
  2. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
  3. Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
Ulcerative colitis
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
  2. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Gout
  1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
Rheumatoid arthritis
  1. Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
Colorectal cancer
  1. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ]
Diverticular disease
  1. Tursi A, Mastromarino P, Capobianco D, Elisei W, Miccheli A, Capuani G, Tomassini A, Campagna G, Picchio M, Giorgetti G, Fabiocchi F, Brandimarte G: Assessment of Fecal Microbiota and Fecal Metabolome in Symptomatic Uncomplicated Diverticular Disease of the Colon. J Clin Gastroenterol. 2016 Oct;50 Suppl 1:S9-S12. doi: 10.1097/MCG.0000000000000626. [PubMed:27622378 ]
Diabetes mellitus type 2
  1. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed:6321058 ]
  2. Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [PubMed:6810706 ]
  3. NA (1999). World Health Organisation Department of Noncommunicable Disease Surveillance (1999). "Definition, Diagnosis and Classification of Diabetes Mellitus and its Complications". WHO.
Diabetes mellitus type 1
  1. Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU (2010). Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037 . Rev. Roum. Chim.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
  • 130650 (Beckwith-Wiedemann Syndrome)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 256450 (Hyperinsulinemic hypoglycemia, familial, 1, HHF1)
  • 603376 (Long-chain Fatty Acids, Defect in Transport of)
  • 212140 (Carnitine transporter defect; primary systemic carnitine deficiency)
  • 229700 (Fructose-1,6-diphosphatase deficiency)
  • 256000 (Leigh's syndrome, subacute necrotizing encephalopathy, SNE)
  • 600955 (Proprotein Convertase 1/3 Deficiency)
  • 520000 (Diabetes and Deafness, Maternally Inherited)
  • 605911 (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
  • 248250 (Primary hypomagnesemia)
  • 614962 (Leptin Deficiency or Dysfunction)
  • 201910 (21-Hydroxylase deficiency)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 255120 (Carnitine palmitoyltransferase I deficiency)
  • 602439 (Acute myelogenous leukemia)
  • 609015 (Mitochondrial trifunctional protein deficiency)
  • 240800 (Hypoglycemia, familial neonatal)
  • 238600 (Hyperlipoproteinemia)
  • 300438 (2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency)
  • 139250 (Growth hormone deficiency)
  • 613743 (Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete)
  • 125851 (Maturity onset diabetes of the young, type 2)
  • 181500 (Schizophrenia)
  • 201810 (Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency)
  • 616483 (Infantile Liver Failure Syndrome 2)
  • 616878 (Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration)
  • 246200 (Donohue Syndrome)
  • 261680 (Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic)
  • 608594 (Lipodystrophy, Congenital Generalized)
  • 608600 (Familial partial lipodystrophy)
  • 210200 (3-Methyl-crotonyl-glycinuria)
  • 240200 (Addison's Disease)
  • 227810 (Fanconi Bickel syndrome)
  • 226980 (Wolcott-Rallison syndrome)
  • 104300 (Alzheimer's disease)
  • 606777 (Glucose transporter type 1 deficiency syndrome)
  • 138900 (Gout)
  • 180300 (Rheumatoid arthritis)
  • 114500 (Colorectal cancer)
  • 125853 (Diabetes mellitus type 2)
  • 222100 (Diabetes mellitus type 1)
  • 610247 (Eosinophilic esophagitis)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB011829
KNApSAcK IDC00001122
Chemspider ID71358
KEGG Compound IDC00267
BioCyc IDALPHA-GLUCOSE
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6897
PubChem Compound79025
PDB IDNot Available
ChEBI ID17925
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceSimmons, Blake A.; Volponi, Joanne V.; Ingersoll, David; Walker, Andrew. Conversion of sucrose to b-D-glucose using three-stage immobilized enzyme process. U.S. (2007), 12pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Onyshchenko AM, Korobkova KS, Kovalenko NK, Kasumova SO, Skrypal' IH: [The role of the carbohydrate composition of the glycocalyx in some species of lactobacilli in the manifestation of their adhesive properties]. Mikrobiol Z. 1999 Nov-Dec;61(6):22-8. [PubMed:10733280 ]
  2. Georgiou S, Pasmatzi E, Monastirli A, Sakkis T, Alachioti S, Tsambaos D: Age-related alterations in the carbohydrate residue composition of the cell surface in the unexposed normal human epidermis. Gerontology. 2005 May-Jun;51(3):155-60. [PubMed:15832040 ]
  3. Skrypal' IH, Tokovenko IP, Malynovs'ka LP: [Carbohydrate receptors for Mycoplasma fermentans adhesion on human epithelial tissues]. Mikrobiol Z. 1995 Jul-Aug;57(4):17-22. [PubMed:8548067 ]
  4. Dalmau SR, Freitas CS: Sugar inhibition of the lectin jacalin: comparison of three assays. Braz J Med Biol Res. 1989;22(5):601-10. [PubMed:2620170 ]
  5. Van Cleve JW: Improved preparation of methyl 4,6-O-benzylidene-alpha-d-glucopyranoside. Carbohydr Res. 1971 Apr;17(2):461-4. [PubMed:5150910 ]
  6. Yariv J, Kalb AJ, Levitzki A: The interaction of concanavalin A with methyl alpha-D-glucopyranoside. Biochim Biophys Acta. 1968 Sep 3;165(2):303-5. [PubMed:5683533 ]
  7. Binder TP, Robyt JF: p-Nitrophenyl alpha-D-glucopyranoside, a new substrate for glucansucrases. Carbohydr Res. 1983 Dec 23;124(2):287-99. [PubMed:6671201 ]
  8. Farnback M, Eriksson L, Widmalm G: Methyl 3-O-beta-L-fucopyranosyl alpha-D-glucopyranoside trihydrate. Acta Crystallogr C. 2003 Mar;59(Pt 3):o171-3. Epub 2003 Feb 28. [PubMed:12711799 ]
  9. Pan Q, Noll BC, Serianni AS: Methyl 4-O-beta-D-galactopyranosyl alpha-D-glucopyranoside (methyl alpha-lactoside). Acta Crystallogr C. 2005 Dec;61(Pt 12):o674-7. Epub 2005 Nov 11. [PubMed:16330845 ]
  10. Singh VP, Bali A, Singh N, Jaggi AS: Advanced glycation end products and diabetic complications. Korean J Physiol Pharmacol. 2014 Feb;18(1):1-14. doi: 10.4196/kjpp.2014.18.1.1. Epub 2014 Feb 13. [PubMed:24634591 ]
  11. Freckmann G, Hagenlocher S, Baumstark A, Jendrike N, Gillen RC, Rossner K, Haug C: Continuous glucose profiles in healthy subjects under everyday life conditions and after different meals. J Diabetes Sci Technol. 2007 Sep;1(5):695-703. doi: 10.1177/193229680700100513. [PubMed:19885137 ]
  12. Butler SO, Btaiche IF, Alaniz C: Relationship between hyperglycemia and infection in critically ill patients. Pharmacotherapy. 2005 Jul;25(7):963-76. doi: 10.1592/phco.2005.25.7.963. [PubMed:16006275 ]
  13. Christiane Godl, Thornthan Sawangwan, Bernd Nidetzky, Mario Muller, 'Method For Producing 2-O-Glyceryl-Alpha-D-Glucopyranoside.' U.S. Patent US20090318372, issued December 24, 2009. [Link]
  14. Wan S. Kim, Seo Y. Jeong, James C. McRea, 'Amido-phenyl-.alpha.-D-glucopyranoside derivatives.' U.S. Patent US4536572, issued August, 1983. [Link]
  15. Shoji Usami, Kohtaro Kirimura, Hiroyuki Nakagawa, Yukio Dobashi, Masaaki Yoshiyama, Susumu Shimura, Yoshio Ito, 'Method of producing 1-menthyl- .alpha.-D-glucopyranoside.' U.S. Patent US6277602, issued September, 1996. [Link]

Only showing the first 10 proteins. There are 37 proteins in total.

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the NADPH-dependent reduction of a wide variety of carbonyl-containing compounds to their corresponding alcohols with a broad range of catalytic efficiencies.
Gene Name:
AKR1B1
Uniprot ID:
P15121
Molecular weight:
35853.125
Reactions
Sorbitol + NADP → alpha-D-Glucose + NADPH + Hydrogen Iondetails
General function:
Involved in catalytic activity
Specific function:
Involved primarily in ATP hydrolysis at the plasma membrane. Plays a role in regulating pyrophosphate levels, and functions in bone mineralization and soft tissue calcification. In vitro, has a broad specificity, hydrolyzing other nucleoside 5' triphosphates such as GTP, CTP, TTP and UTP to their corresponding monophosphates with release of pyrophosphate and diadenosine polyphosphates, and also 3',5'-cAMP to AMP. May also be involved in the regulation of the availability of nucleotide sugars in the endoplasmic reticulum and Golgi, and the regulation of purinergic signaling. Appears to modulate insulin sensitivity.
Gene Name:
ENPP1
Uniprot ID:
P22413
Molecular weight:
104923.58
General function:
Involved in ATP binding
Specific function:
Catalyzes the initial step in utilization of glucose by the beta-cell and liver at physiological glucose concentration. Glucokinase has a high Km for glucose, and so it is effective only when glucose is abundant. The role of GCK is to provide G6P for the synthesis of glycogen. Pancreatic glucokinase plays an important role in modulating insulin secretion. Hepatic glucokinase helps to facilitate the uptake and conversion of glucose by acting as an insulin-sensitive determinant of hepatic glucose usage.
Gene Name:
GCK
Uniprot ID:
P35557
Molecular weight:
52191.07
Reactions
Adenosine triphosphate + alpha-D-Glucose → ADP + Glucose 6-phosphatedetails
General function:
Involved in ATP binding
Specific function:
Not Available
Gene Name:
HK3
Uniprot ID:
P52790
Molecular weight:
99024.56
Reactions
Adenosine triphosphate + alpha-D-Glucose → ADP + Glucose 6-phosphatedetails
General function:
Involved in ATP binding
Specific function:
Not Available
Gene Name:
HK2
Uniprot ID:
P52789
Molecular weight:
102379.06
Reactions
Adenosine triphosphate + alpha-D-Glucose → ADP + Glucose 6-phosphatedetails
General function:
Involved in ATP binding
Specific function:
Not Available
Gene Name:
HK1
Uniprot ID:
P19367
Molecular weight:
102485.1
Reactions
Adenosine triphosphate + alpha-D-Glucose → ADP + Glucose 6-phosphatedetails
General function:
Involved in catalytic activity
Specific function:
Plays an important role in the final stage of carbohydrate digestion. Isomaltase activity is specific for both alpha-1,4- and alpha-1,6-oligosaccharides.
Gene Name:
SI
Uniprot ID:
P14410
Molecular weight:
Not Available
Reactions
Sucrose + Water → D-Fructose + alpha-D-Glucosedetails
Isomaltose + Water → alpha-D-Glucose + D-Glucosedetails
Dextrin + Water → alpha-D-Glucose + Dextrindetails
Neohancoside D + Water → D-Fructose + alpha-D-Glucosedetails
+ Water → alpha-D-Glucose + details
General function:
Involved in 6-phosphogluconolactonase activity
Specific function:
Oxidizes glucose-6-phosphate and glucose, as well as other hexose-6-phosphates.
Gene Name:
H6PD
Uniprot ID:
O95479
Molecular weight:
88891.99
General function:
Involved in hydrolase activity, hydrolyzing O-glycosyl compounds
Specific function:
LPH splits lactose in the small intestine.
Gene Name:
LCT
Uniprot ID:
P09848
Molecular weight:
218584.77
Reactions
beta-Lactose + Water → alpha-D-Glucose + beta-D-Galactosedetails
General function:
Involved in catalytic activity
Specific function:
Cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor of immature glycoproteins.
Gene Name:
GANAB
Uniprot ID:
Q14697
Molecular weight:
Not Available

Only showing the first 10 proteins. There are 37 proteins in total.