Human Metabolome Database: Showing metabocard for L-Valine (HMDB0000883)

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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (6)transporters (1) Show 7 proteins XML

enzymes (6)transporters (1) Show 7 proteins

Record Information

Version

4.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2019-01-11 19:15:15 UTC

HMDB ID

HMDB0000883

Secondary Accession Numbers

HMDB0034366
HMDB00883
HMDB34366

Metabolite Identification

Common Name

L-Valine

Description

Valine (abbreviated as Val or V) is an -amino acid with the chemical formula HO2CCH(NH2)CH(CH3)2. It is named after the plant valerian. L-Valine is one of 20 proteinogenic amino acids. Its codons are GUU, GUC, GUA, and GUG. This essential amino acid is classified as nonpolar. Along with leucine and isoleucine, valine is a branched-chain amino acid. Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)â€”tyrosine, tryptophan and phenylalanine, as well as methionineâ€”are increased in these conditions. Valine in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio (http://www.dcnutrition.com). In sickle-cell disease, valine substitutes for the hydrophilic amino acid glutamic acid in hemoglobin. Because valine is hydrophobic, the hemoglobin does not fold correctly. Hypervalinemia is another example of an inborn error of metabolism involving Valine. Valine is an essential amino acid, hence it must be ingested, usually as a component of proteins.

Structure

MOL SDF 3D-SDF PDB SMILES InChI View 3D Structure

Synonyms

Value	Source
(2S)-2-amino-3-Methylbutanoic acid	ChEBI
(S)-Valine	ChEBI
2-amino-3-Methylbutyric acid	ChEBI
L-(+)-alpha-Aminoisovaleric acid	ChEBI
L-alpha-amino-beta-Methylbutyric acid	ChEBI
L-Valin	ChEBI
V	ChEBI
Val	ChEBI
VALINE	ChEBI
(2S)-2-amino-3-Methylbutanoate	Generator
2-amino-3-Methylbutyrate	Generator
L-(+)-a-Aminoisovalerate	Generator
L-(+)-a-Aminoisovaleric acid	Generator
L-(+)-alpha-Aminoisovalerate	Generator
L-(+)-α-aminoisovalerate	Generator
L-(+)-α-aminoisovaleric acid	Generator
L-a-amino-b-Methylbutyrate	Generator
L-a-amino-b-Methylbutyric acid	Generator
L-alpha-amino-beta-Methylbutyrate	Generator
L-α-amino-β-methylbutyrate	Generator
L-α-amino-β-methylbutyric acid	Generator
(S)-2-amino-3-Methyl-butanoate	HMDB
(S)-2-amino-3-Methyl-butanoic acid	HMDB
(S)-2-amino-3-Methylbutanoate	HMDB
(S)-2-amino-3-Methylbutanoic acid	HMDB
(S)-2-amino-3-Methylbutyrate	HMDB
(S)-2-amino-3-Methylbutyric acid	HMDB
(S)-a-amino-b-Methylbutyrate	HMDB
(S)-a-amino-b-Methylbutyric acid	HMDB
(S)-alpha-amino-beta-Methylbutyrate	HMDB
(S)-alpha-amino-beta-Methylbutyric acid	HMDB
2-amino-3-Methylbutanoate	HMDB
2-amino-3-Methylbutanoic acid	HMDB
L Valine	MeSH

Chemical Formula

C₅H₁₁NO₂

Average Molecular Weight

117.1463

Monoisotopic Molecular Weight

117.078978601

IUPAC Name

(2S)-2-amino-3-methylbutanoic acid

Traditional Name

L-valine

CAS Registry Number

72-18-4

SMILES

CC(C)[C@H](N)C(O)=O

InChI Identifier

InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1

InChI Key

KZSNJWFQEVHDMF-BYPYZUCNSA-N

Chemical Taxonomy

Description

This compound belongs to the class of organic compounds known as valine and derivatives. These are compounds containing valine or a derivative thereof resulting from reaction of valine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Valine and derivatives

Alternative Parents

Substituents

Valine or derivatives
Alpha-amino acid
L-alpha-amino acid
Branched fatty acid
Methyl-branched fatty acid
Fatty acid
Fatty acyl
Amino acid
Monocarboxylic acid or derivatives
Carboxylic acid
Organic oxide
Organopnictogen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Carbonyl group
Organic oxygen compound
Amine
Organic nitrogen compound
Hydrocarbon derivative
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

proteinogenic amino acid (CHEBI:16414 )
L-alpha-amino acid (CHEBI:16414 )
valine (CHEBI:16414 )
pyruvate family amino acid (CHEBI:16414 )
Common amino acids (C00183 )

Ontology

Physiological effect

Health effect:

Health condition:

Metabolism and nutrition disorders:

Hypoglycemia

Ingestion

Source:

Food

Synthetic:

Personal care product

Biological:

Animal

Plant:

Biological location:

Tissue and substructures:

Epidermis

Biofluid and excreta:

Cell and elements:

Extracellular

Cell:

Fibroblast

Subcellular:

Process

Naturally occurring process:

Biological process:

Biochemical pathway:

Role

Industrial application:

Personal care products

Food and nutrition:

Nutritional supplement

Pharmaceutical industry:

Pharmaceutical

Biological role:

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	295 - 300 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	58.5 mg/mL	Not Available
LogP	-2.26	HANSCH,C ET AL. (1995)

Predicted Properties

Property	Value	Source
Water Solubility	214 g/L	ALOGPS
logP	-2.3	ALOGPS
logP	-2	ChemAxon
logS	0.26	ALOGPS
pKa (Strongest Acidic)	2.72	ChemAxon
pKa (Strongest Basic)	9.6	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	63.32 Å²	ChemAxon
Rotatable Bond Count	2	ChemAxon
Refractivity	29.49 m³·mol⁻¹	ChemAxon
Polarizability	12.19 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	1	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	Yes	ChemAxon
Veber's Rule	Yes	ChemAxon
MDDR-like Rule	Yes	ChemAxon

Spectra

Spectrum Type	Description	Splash Key
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0006-0910000000-23bfcf2795f71f458bda	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0006-0920000000-7892bb9d4d9afcc23410	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0006-0910000000-f2491152816b9c5dc7ed	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (1 TMS)	splash10-0znj-1900000000-7c976d7c11a402db399f	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-00dl-8910000000-78e8ee971ad7298c23cd	View in MoNA
GC-MS	GC-MS Spectrum - GC-MS (1 TMS)	splash10-00di-9200000000-805e6724e5181af78c81	View in MoNA
GC-MS	GC-MS Spectrum - GC-MS (2 TMS)	splash10-0006-0910000000-a212771a5ea868139d5a	View in MoNA
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-059t-0900000000-7b8e4e98ac9158bf26d9	View in MoNA
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-0006-0910000000-9079324093be9a14d47a	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0910000000-23bfcf2795f71f458bda	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0920000000-7892bb9d4d9afcc23410	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0910000000-f2491152816b9c5dc7ed	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0znj-1900000000-7c976d7c11a402db399f	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-QQ (Non-derivatized)	splash10-000i-2920000000-1628d46d5520c5f20de8	View in MoNA
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00dl-8910000000-78e8ee971ad7298c23cd	View in MoNA
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00di-9200000000-805e6724e5181af78c81	View in MoNA
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0006-0910000000-a212771a5ea868139d5a	View in MoNA
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00dl-9000000000-602d1e01bc7567493e4c	View in MoNA
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-00dl-9500000000-a758682a7aafbd0d553e	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-00di-9000000000-67498a3295a1fe788c4b	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-0a4i-9000000000-fb359f44fcf1b83ee14d	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0a4i-9000000000-8e5f620c04615765a062	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014r-0900000000-619d80cc41221405e0bd	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-00di-9000000000-17ab53cec2e6b727c3e9	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-00di-9000000000-0dfbcc85d6423af91eda	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014l-4900000000-c50d79ba3a5013bcf29f	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-0uxr-0900000000-67a7784963ec75cecaa0	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-00di-9000000000-4569516f23d8a712b434	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-d928cac1226f19b8edc3	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-0udi-0900000000-34059351f2cbd94a8acc	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-014i-0900000000-a36d8d68a6705027415f	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-014i-0900000000-1a5d8f4c1b293de18fed	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-014i-0900000000-b410df2c18dfc8ab7165	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-0gi0-6900000000-c87ff036e3a5d15b7670	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-00di-9000000000-561b3b28fa82e0b10658	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-05fr-9000000000-11e7c0a3d5cf8e4dedfe	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-0a4i-9000000000-6a6f8cd9629b0f09469c	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-0a4i-9000000000-4518f691bbda5739c0f1	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive	splash10-014i-0900000000-a931cfc8207edac33775	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-00di-9000000000-430332b13b8c6368136b	View in MoNA
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-014i-0900000000-ffb286a8ae38a0a3e6d2	View in MoNA
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-00xr-9500000000-bb54662a5fd1dd142d90	View in MoNA
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-00di-9000000000-1f7d9c10db4226a383c1	View in MoNA
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-05fu-9000000000-f6d7e45bb3f3436e5eb7	View in MoNA
MS	Mass Spectrum (Electron Ionization)	splash10-05fr-9000000000-5d10fb7acd70b2f43368	View in MoNA
1D NMR	13C NMR Spectrum	Not Available	View in JSpectraViewer
1D NMR	1H NMR Spectrum	Not Available	View in JSpectraViewer
1D NMR	1H NMR Spectrum	Not Available	View in JSpectraViewer
2D NMR	[1H,1H] 2D NMR Spectrum	Not Available	View in JSpectraViewer
2D NMR	[1H,13C] 2D NMR Spectrum	Not Available	View in JSpectraViewer

Biological Properties

Cellular Locations

Extracellular
Mitochondria

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

Epidermis
Fibroblasts

Pathways

Name	SMPDB/Pathwhiz	KEGG
Valine, Leucine and Isoleucine Degradation
Propanoate Metabolism
Transcription/Translation	SMP00019	Not Available
Beta-Ketothiolase Deficiency		Not Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	212.3 +/- 61.3 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	266.3 +/- 61 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	75-265 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	71-280 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	65-325 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	115-385 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	74-321 uM	Children (1 - 18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	233.0 (190.0-276.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	238 (206-272) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	130.0 +/- 50.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	232.0 +/- 28.0 uM	Children (1-13 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	252.0 +/- 37.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	209.0 +/- 31.0 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	142-278 uM	Adolescent (13-18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	158-310 uM	Infant (0-1 year old)	Not Specified	Normal	24341803	details
Blood	Detected and Quantified	119-336 uM	Adult (>18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	133-273 uM	Children (1-13 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	257 +/- 39 uM	Adult (>18 years old)	Male	Normal	2415198	details
Blood	Detected and Quantified	210 +/- 19 uM	Adult (>18 years old)	Female	Normal	2415198	details
Blood	Detected and Quantified	248 (206-304) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	260.7 +/- 39.7 uM	Children (1 - 13 years old)	Not Specified	Normal	18088602	details
Blood	Detected and Quantified	209 +/- 31 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	243.6 +/- 45.2 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	252 +/- 37 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	130.9(114.6-157.3) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	181 +/- 20 uM	Children (1 - 13 years old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	140.0-300.0 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Breast Milk	Detected and Quantified	48.7 +/- 11.2 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	20.0 (10.0-30.0) uM	Adult (>18 years old)	Both	Normal	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	19 +/- 13 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	15.0 +/- 2.8 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	23.8 +/- 4.5 uM	Adult (>18 years old)	Not Specified	Normal	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	23.9 +/- 6.9 uM	Adult (>18 years old)	Male	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	14.5 +/- 5.0 uM	Adult (>18 years old)	Female	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	18.1 +/- 10.1 uM	Adult (>18 years old)	Not Specified	Normal	22546835	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	7.5-23 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4-32 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Feces	Detected and Quantified	4719 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified		Adult (>18 years old)	Male	Normal	28270806	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25670064	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	23940645	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified		Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified		Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected but not Quantified		Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected and Quantified	66298.864 (26775.636-105822.0931) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	46352.296 (19804.296-72900.296) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	68091.495 (36080.240-100102.749) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected but not Quantified		Not Specified	Not Specified	Normal	22944170	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	24029555	details
Feces	Detected but not Quantified		Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	Normal	Christopher ...	details
Feces	Detected and Quantified	49.596 +/- 55.0594 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	Normal	26709303	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected but not Quantified		Infant (0-1 year old)	Both	Normal	24375085	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected and Quantified	1440 +/- 810 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	2023 +/- 842 nmol/g wet feces	Adult (>18 years old)	Female	Normal	23997757	details
Feces	Detected but not Quantified		Adult (>18 years old)	Not Specified	Normal	27622378	details
Feces	Detected and Quantified	250 +/- 230 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	27107423	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	17269711	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected and Quantified	4408 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25598765	details
Feces	Detected and Quantified	4027 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified		Not Specified	Not Specified	Normal	20669995	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Normal	21190195	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected and Quantified	11.3 +/- 9.4 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	16.2 +/- 12.3 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	6.3 +/- 5.4 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	3.63 +/- 1.66 uM	Adult (>18 years old)	Both	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	47.97 +/- 33.60 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	10.2 +/- 8 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	8.45 +/- 6.82 uM	Adult (>18 years old)	Female	Normal	23857558	details
Sweat	Detected and Quantified	110-430 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	70 uM	Adult (40 years old)	Male	Normal	22194922	details
Sweat	Detected but not Quantified		Adult	Both	Normal	26755145	details
Urine	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22932811	details
Urine	Detected and Quantified	5.5 (2.7-9.8) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	4.93 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	22800120	details
Urine	Detected and Quantified	12.30 +/- 7.69 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	2026685	details
Urine	Detected and Quantified	<5.98-25.62 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	1.7-3.4 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	2.327 +/- 1.220 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	1.373 +/- 1.620 umol/mmol creatinine	Children (1 - 13 years old)	Female	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	3.113 +/- 1.0400 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	2.88 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	1.700 +/- 0.813 umol/mmol creatinine	Children (1 - 13 years old)	Male	Normal	Geigy Scientific ...	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.0177 - 0.0353 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	6.9 +/- 3.858 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	3.15 +/- 1.49 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	4.3 (2.4-10.4) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	11.58 (8.51 – 15.04) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Lorena Ivona ŞTEF...	details
Urine	Detected and Quantified	3.4 (2.0-7.7) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.8 (0.3-1.35) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	1.67 +/- 0.80 umol/mmol creatinine	Children (1-13 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	3.17 - 24.42 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	3.0 +/- 1.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	4.07 - 17.98 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	2.3 +/- 1.2 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	5.99 - 17.98 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	6.90 - 19.79 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	5.99 - 18.88 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	3.28 - 16.05 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	3.28 - 12.44 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	3.28 - 12.44 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	1.9-2.9 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	2 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	3.355(1.118-5.592) umol/mmol creatinine	Adult (>18 years old)	Both	Normal		details
Urine	Detected and Quantified	9.3735 +/- 3.2488 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected but not Quantified		Adult (>18 years old)	Both	Oesophageal cancer	22291914	details
Blood	Detected and Quantified	117.1 (33.4) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	114.8 (34.9) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Blood	Detected and Quantified	844 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Blood	Detected and Quantified	247.7 +/- 72.7 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	278.1 +/- 62.4 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	144.2 +/- 61.4 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected and Quantified	81.0 +/- 122.0 uM	Newborn (0-30 days old)	Both	Phenylketonuria	12101068	details
Blood	Detected and Quantified	193.0 (184.0-201.0) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	202.0 (194.0-209.0) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	356.0 +/- 541.0 uM	Newborn (0-30 days old)	Both	Maple syrup urine disease (MSUD)	12101068	details
Blood	Detected and Quantified	121.9 +/- 44.00 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	119.6 +/- 38.1 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	119.1 (29.5) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	129.8 (51.1) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected but not Quantified		Adult (>18 years old)	Both	oesophago-gastric cancer	23896378	details
Blood	Detected and Quantified	657.298 uM	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	6688766	details
Blood	Detected and Quantified	44-89 uM	Adolescent (13-18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	128 (32-155) uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Blood	Detected and Quantified	57-86 uM	Adult (>18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	32-147.300 uM	Children (1-13 years old)	Both	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	273 +/- 45 uM	Adult (>18 years old)	Male	Schizophrenia	2415198	details
Blood	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	20156336	details
Blood	Detected and Quantified	232 +/- 44 uM	Adult (>18 years old)	Female	Schizophrenia	2415198	details
Blood	Detected and Quantified	1495-1675 uM	Children (1 - 13 years old)	Both	Hypervalinemia	15168722	details
Blood	Detected and Quantified	197.7 +/- 184.2 uM	Children (1 - 13 years old)	Not Specified	Maple syrup urine disease	18088602	details
Blood	Detected and Quantified	146.4(125.0-176.6) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	142.5 (50.6) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	121.6 (43.3) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected and Quantified	58.26 +/- 14.74 uM	Elderly (>65 years old)	Both	Alzheimer's disease	17031479	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	359 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	98.61 uM	Adult (>18 years old)	Not Specified	Cachexia	18953024	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	21.8 +/- 7.2 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	17.9 +/- 6.7 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.13 +/- 1.22 uM	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified		Adult (>18 years old)	Male	Gout	28270806	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Immunoglobulin A nephropathy (IgAN) progressor	26476344	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	ankylosing spondylitis	Tie-juan Sha...	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	rheumatoid arthritis	Tie-juan Sha...	details
Feces	Detected but not Quantified		Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified		Not Specified	Not Specified	Cryptosporidium infection	22944170	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified		Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	Myalgic encephalomyelitis/chronic fatigue syndrome	Christopher ...	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Irritable Bowel Syndrome	21494186	details
Feces	Detected but not Quantified		Adult (>18 years old)	Not Specified	asymptomatic diverticulosis	27622378	details
Feces	Detected but not Quantified		Adult (>18 years old)	Not Specified	symptomatic uncomplicated diverticular disease	27622378	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal Cancer	27107423	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Crohn’s Disease	17269711	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Ulcerative Colitis	17269711	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Crohn's disease	17269711	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Crohns disease	25598765	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Ulcerative colitis	25598765	details
Saliva	Detected and Quantified	4.93 +/- 2.31 uM	Adult (>18 years old)	Both	Oral cavity disorders	Sugimoto et al. (...	details
Saliva	Detected and Quantified	4.27 +/- 0.46 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Oral squamous cell carcinoma (OSCC)	21190195	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Oral leukoplakia (OLK)	21190195	details
Saliva	Detected and Quantified	4.77 +/- 1.15 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Oral cancer	20300169	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Female	Breast cancer	20300169	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Not Specified	Pancreatic cancer	20300169	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Not Specified	Periodontal diseases	20300169	details
Saliva	Detected and Quantified	5.55 +/- 3.92 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Schizophrenia	22800120	details
Urine	Detected and Quantified	18.246-18.720 umol/mmol creatinine	Children (1 - 13 years old)	Both	Hypervalinemia	15168722	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Autosomal dominant polycystic kidney disease (ADPKD)	21389975	details
Urine	Detected and Quantified	8.017 +/- 7.53 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	3 (0-14) umol/mmol creatinine	Adult (>18 years old)	Both	Lung cancer	22157537	details
Urine	Detected and Quantified	15.49 (4.79 – 30.71) umol/mmol creatinine	Adult (>18 years old)	Both	Type 1 diabetes Mellitus	Lorena Ivona ŞTEF...	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	oesophago-gastric cancer	23896378	details
Urine	Detected and Quantified	0.73 +/- 0.12 umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Schizophrenia	22024767	details
Urine	Detected and Quantified	32.4 umol/mmol creatinine	Adult (>18 years old)	Both	Paraquat poisoning	9625050	details
Urine	Detected and Quantified	7.6407 +/- 4.4532 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	6.9736 +/- 3.7288 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Mordechai, Hien, ...	details

Associated Disorders and Diseases

Disease References

Phenylketonuria
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Epilepsy
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Maple syrup urine disease
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ] Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ] Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Alzheimer's disease
Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ] Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Schizophrenia
Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ] 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 ] 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 ]
Early preeclampsia
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
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 ] 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 ] 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 ] 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 ]
Late-onset preeclampsia
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 ]
Hypervalinemia
Swarna M, Jyothy A, Usha Rani P, Reddy PP: Amino acid disorders in mental retardation: a two-decade study from Andhra Pradesh. Biochem Genet. 2004 Apr;42(3-4):85-98. [PubMed:15168722 ]
Obesity
Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Dihydrolipoamide Dehydrogenase Deficiency
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Lipoyltransferase 1 Deficiency
Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Cachexia
Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 2008 Oct 25. [PubMed:18953024 ]
Leukemia
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Crohn's disease
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] 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 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Irritable bowel syndrome
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 ] 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 ]
Colorectal cancer
Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ] Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ] Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] 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 ] Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ] 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 ] 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 ]
Autism
De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Diverticular disease
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 ]
Gout
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
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]
Perillyl alcohol administration for cancer treatment
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Periodontal disease
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Paraquat poisoning
Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. [PubMed:9625050 ]
Lung Cancer
Stretch C, Eastman T, Mandal R, Eisner R, Wishart DS, Mourtzakis M, Prado CM, Damaraju S, Ball RO, Greiner R, Baracos VE: Prediction of skeletal muscle and fat mass in patients with advanced cancer using a metabolomic approach. J Nutr. 2012 Jan;142(1):14-21. doi: 10.3945/jn.111.147751. Epub 2011 Dec 7. [PubMed:22157537 ]
Diabetes mellitus type 1
(). 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 . .
Autosomal dominant polycystic kidney disease
Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .

Associated OMIM IDs

261600 (Phenylketonuria)
248600 (Maple syrup urine disease)
104300 (Alzheimer's disease)
181500 (Schizophrenia)
277100 (Hypervalinemia)
601665 (Obesity)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)
614923 (Branched-chain Keto Acid Dehydrogenase Kinase Deficiency)
616299 (Lipoyltransferase 1 Deficiency)
266600 (Crohn's disease)
114500 (Colorectal cancer)
209850 (Autism)
138900 (Gout)
180300 (Rheumatoid arthritis)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
600274 (Frontotemporal dementia)
211980 (Lung Cancer)
222100 (Diabetes mellitus type 1)
601313 (Autosomal dominant polycystic kidney disease)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

DB00161

Phenol Explorer Compound ID

Not Available

FoodDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

PDB ID

ChEBI ID

References

Synthesis Reference

Kinoshita, Shukuo; Udaka, Shigezo. L-Valine production by fermentation. (1962), 2 pp.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. [PubMed:9625050 ]
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Stickel F, Osterreicher CH, Datz C, Ferenci P, Wolfel M, Norgauer W, Kraus MR, Wrba F, Hellerbrand C, Schuppan D: Prediction of progression to cirrhosis by a glutathione S-transferase P1 polymorphism in subjects with hereditary hemochromatosis. Arch Intern Med. 2005 Sep 12;165(16):1835-40. [PubMed:16157826 ]
Wudy SA, Hartmann M, Solleder C, Homoki J: Determination of 17alpha-hydroxypregnenolone in human plasma by routine isotope dilution mass spectrometry using benchtop gas chromatography-mass selective detection. Steroids. 2001 Oct;66(10):759-62. [PubMed:11522338 ]
Szpetnar M, Pasternak K, Boguszewska A: Branched chain amino acids (BCAAs) in heart diseases (ischaemic heart disease and myocardial infarction). Ann Univ Mariae Curie Sklodowska Med. 2004;59(2):91-5. [PubMed:16146056 ]
Hongpaisan J: Inhibition of proliferation of contaminating fibroblasts by D-valine in cultures of smooth muscle cells from human myometrium. Cell Biol Int. 2000;24(1):1-7. [PubMed:10826768 ]
Deligezer U, Akisik EE, Dalay N: Homozygosity at the C677T of the MTHFR gene is associated with increased breast cancer risk in the Turkish population. In Vivo. 2005 Sep-Oct;19(5):889-93. [PubMed:16097444 ]
McInturff JE, Wang SJ, Machleidt T, Lin TR, Oren A, Hertz CJ, Krutzik SR, Hart S, Zeh K, Anderson DH, Gallo RL, Modlin RL, Kim J: Granulysin-derived peptides demonstrate antimicrobial and anti-inflammatory effects against Propionibacterium acnes. J Invest Dermatol. 2005 Aug;125(2):256-63. [PubMed:16098035 ]
Jensen PK, Jacobsen NO: Studies of D-amino acid oxidase activity in human epidermis and cultured human epidermal cells. Arch Dermatol Res. 1984;276(1):57-64. [PubMed:6142701 ]
Kurpad AV, Regan MM, Raj TD, Gnanou JV, Rao VN, Young VR: The daily valine requirement of healthy adult Indians determined by the 24-h indicator amino acid balance approach. Am J Clin Nutr. 2005 Aug;82(2):373-9. [PubMed:16087981 ]

Enzymes

Enzyme Details

1. Propionyl-CoA carboxylase beta chain, mitochondrial

General function:: Involved in ligase activity
Specific function:: Not Available
Gene Name:: PCCB
Uniprot ID:: P05166
Molecular weight:: 58215.13

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Kim SN, Ryu KH, Lee EH, Kim JS, Hahn SH: Molecular analysis of PCCB gene in Korean patients with propionic acidemia. Mol Genet Metab. 2002 Nov;77(3):209-16. [PubMed:12409268 ]

Enzyme Details

2. Catechol O-methyltransferase

General function:: Involved in magnesium ion binding
Specific function:: Catalyzes the O-methylation, and thereby the inactivation, of catecholamine neurotransmitters and catechol hormones. Also shortens the biological half-lives of certain neuroactive drugs, like L-DOPA, alpha-methyl DOPA and isoproterenol.
Gene Name:: COMT
Uniprot ID:: P21964
Molecular weight:: 30036.77

Enzyme Details

3. Branched-chain-amino-acid aminotransferase, cytosolic

General function:: Involved in catalytic activity
Specific function:: Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:: BCAT1
Uniprot ID:: P54687
Molecular weight:: 38644.77

Reactions

L-Valine + Oxoglutaric acid → alpha-Ketoisovaleric acid + L-Glutamic acid	details

References

Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. [PubMed:17554170 ]
Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. [PubMed:15667998 ]
Saito M, Nishimura K, Wakabayashi S, Kurihara T, Nagata Y: Purification of branched-chain amino acid aminotransferase from Helicobacter pylori NCTC 11637. Amino Acids. 2007 Sep;33(3):445-9. Epub 2006 Nov 2. [PubMed:17077963 ]

Enzyme Details

4. Branched-chain-amino-acid aminotransferase, mitochondrial

General function:: Involved in catalytic activity
Specific function:: Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:: BCAT2
Uniprot ID:: O15382
Molecular weight:: 33776.315

Reactions

L-Valine + Oxoglutaric acid → alpha-Ketoisovaleric acid + L-Glutamic acid	details

Enzyme Details

5. Valine--tRNA ligase

General function:: Involved in nucleotide binding
Specific function:: Not Available
Gene Name:: VARS
Uniprot ID:: P26640
Molecular weight:: 140474.755

Reactions

Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)	details
Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)	details

References

Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. [PubMed:17095543 ]
Laforest MJ, Delage L, Marechal-Drouard L: The T-domain of cytosolic tRNAVal, an essential determinant for mitochondrial import. FEBS Lett. 2005 Feb 14;579(5):1072-8. [PubMed:15710393 ]

Enzyme Details

6. Valine--tRNA ligase, mitochondrial

General function:: Involved in nucleotide binding
Specific function:: Not Available
Gene Name:: VARS2
Uniprot ID:: Q5ST30
Molecular weight:: 102414.65

Reactions

Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)	details
Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)	details

Transporters

Enzyme Details

1. Monocarboxylate transporter 10

General function:: Involved in transmembrane transport
Specific function:: Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:: SLC16A10
Uniprot ID:: Q8TF71
Molecular weight:: 55492.1

References

Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]

Showing metabocard for L-Valine (HMDB0000883)

Structure for HMDB0000883 (L-Valine)

Enzymes

References

Reactions

References

Reactions

Reactions

References

Reactions

Transporters

References