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Showing metabocard for Isovaleric acid (HMDB0000718)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (1) Show 1 proteinXML
enzymes (1) Show 1 protein
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:15:06 UTC
HMDB IDHMDB0000718
Secondary Accession Numbers
  • HMDB00718
Metabolite Identification
Common NameIsovaleric acid
DescriptionIsovaleric acid, is a natural fatty acid found in a wide variety of plants and essential oils. Isovaleric acid is clear colorless liquid that is sparingly soluble in water, but well soluble in most common organic solvents. It has been suggested that isovaleric acid from pilot whales, a species frequently consumed in the Faroe Islands, may be the unusual dietary factor in prolonged gestation in the population of the Faroe Islands. Previous studies suggested that was due to the high intake of n-3 polyunsaturated fatty acids has been, but fatty acid data for eicosapentaenoic acid (EPA) and docosahexanoic acid (DHA) in blood lipids of Faroese and Norwegians was reviewed in terms of the type of fish eaten (mostly lean white fish with DHA much greater than EPA); the popular lean fish, thus, probably provides too little EPA to produce a marked effect on human biochemistry (PMID 2646392 ). Isovaleric acid is found to be associated with isovaleric acidemia, which is an inborn error of metabolism.
Structure
Data?1676999706
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000718 (Isovaleric acid)


  Mrv1652309272007312D          

  7  6  0  0  0  0            999 V2000
   -1.5400   -0.0116    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2546   -0.4241    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5400    0.8134    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8256   -0.4241    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1110   -0.0116    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1110    0.8134    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.6034   -0.4241    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  5  7  1  0  0  0  0
  1  3  2  0  0  0  0
  2  1  1  0  0  0  0
M  END
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3D MOL for HMDB0000718 (Isovaleric acid)

HMDB0000718
     RDKit          3D
Isovaleric acid
 17 16  0  0  0  0  0  0  0  0999 V2000
   -1.1041   -1.0778    0.3055 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2871    0.1879    0.4232 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2598    1.3389    0.2805 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7204    0.3330   -0.6970 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6203   -0.8243   -0.6290 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2697   -1.8835   -1.1694 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8261   -0.7437    0.0167 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4639   -1.9728    0.3877 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7521   -1.0593   -0.5984 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8195   -1.0444    1.1762 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2059    0.1713    1.4212 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0747    1.0227   -0.4193 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6858    1.5468    1.2755 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7843    2.2247   -0.1557 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3232    1.2553   -0.4826 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1875    0.4245   -1.6595 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0782    0.1007    0.5245 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  1  8  1  0
  1  9  1  0
  1 10  1  0
  2 11  1  0
  3 12  1  0
  3 13  1  0
  3 14  1  0
  4 15  1  0
  4 16  1  0
  7 17  1  0
M  END
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3D SDF for HMDB0000718 (Isovaleric acid)


  Mrv1652309272007312D          

  7  6  0  0  0  0            999 V2000
   -1.5400   -0.0116    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2546   -0.4241    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5400    0.8134    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8256   -0.4241    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1110   -0.0116    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1110    0.8134    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.6034   -0.4241    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  5  7  1  0  0  0  0
  1  3  2  0  0  0  0
  2  1  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000718

> <DATABASE_NAME>
hmdb

> <SMILES>
CC(C)CC(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C5H10O2/c1-4(2)3-5(6)7/h4H,3H2,1-2H3,(H,6,7)

> <INCHI_KEY>
GWYFCOCPABKNJV-UHFFFAOYSA-N

> <FORMULA>
C5H10O2

> <MOLECULAR_WEIGHT>
102.1317

> <EXACT_MASS>
102.068079564

> <JCHEM_ACCEPTOR_COUNT>
2

> <JCHEM_ATOM_COUNT>
17

> <JCHEM_AVERAGE_POLARIZABILITY>
10.986666365215886

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
3-methylbutanoic acid

> <ALOGPS_LOGP>
1.26

> <JCHEM_LOGP>
1.208777894

> <ALOGPS_LOGS>
-0.20

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA_STRONGEST_ACIDIC>
5.01023295557517

> <JCHEM_POLAR_SURFACE_AREA>
37.3

> <JCHEM_REFRACTIVITY>
26.4202

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
6.49e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
isovaleric acid

> <JCHEM_VEBER_RULE>
1

$$$$
Download

3D-SDF for HMDB0000718 (Isovaleric acid)

HMDB0000718
     RDKit          3D
Isovaleric acid
 17 16  0  0  0  0  0  0  0  0999 V2000
   -1.1041   -1.0778    0.3055 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2871    0.1879    0.4232 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2598    1.3389    0.2805 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7204    0.3330   -0.6970 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6203   -0.8243   -0.6290 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2697   -1.8835   -1.1694 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8261   -0.7437    0.0167 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4639   -1.9728    0.3877 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7521   -1.0593   -0.5984 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8195   -1.0444    1.1762 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2059    0.1713    1.4212 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0747    1.0227   -0.4193 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6858    1.5468    1.2755 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7843    2.2247   -0.1557 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3232    1.2553   -0.4826 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1875    0.4245   -1.6595 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0782    0.1007    0.5245 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  1  8  1  0
  1  9  1  0
  1 10  1  0
  2 11  1  0
  3 12  1  0
  3 13  1  0
  3 14  1  0
  4 15  1  0
  4 16  1  0
  7 17  1  0
M  END
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PDB for HMDB0000718 (Isovaleric acid)

HEADER    PROTEIN                                 27-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-SEP-20         0                                  
HETATM    1  C   UNK     0      -2.875  -0.022   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0      -4.209  -0.792   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0      -2.875   1.518   0.000  0.00  0.00           O+0
HETATM    4  C   UNK     0      -1.541  -0.792   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      -0.207  -0.022   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      -0.207   1.518   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0       1.126  -0.792   0.000  0.00  0.00           C+0
CONECT    1    4    3    2                                                  
CONECT    2    1                                                            
CONECT    3    1                                                            
CONECT    4    1    5                                                       
CONECT    5    4    6    7                                                  
CONECT    6    5                                                            
CONECT    7    5                                                            
MASTER        0    0    0    0    0    0    0    0    7    0   12    0
END
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3D PDB for HMDB0000718 (Isovaleric acid)

COMPND    HMDB0000718
HETATM    1  C1  UNL     1      -1.104  -1.078   0.305  1.00  0.00           C  
HETATM    2  C2  UNL     1      -0.287   0.188   0.423  1.00  0.00           C  
HETATM    3  C3  UNL     1      -1.260   1.339   0.281  1.00  0.00           C  
HETATM    4  C4  UNL     1       0.720   0.333  -0.697  1.00  0.00           C  
HETATM    5  C5  UNL     1       1.620  -0.824  -0.629  1.00  0.00           C  
HETATM    6  O1  UNL     1       1.270  -1.883  -1.169  1.00  0.00           O  
HETATM    7  O2  UNL     1       2.826  -0.744   0.017  1.00  0.00           O  
HETATM    8  H1  UNL     1      -0.464  -1.973   0.388  1.00  0.00           H  
HETATM    9  H2  UNL     1      -1.752  -1.059  -0.598  1.00  0.00           H  
HETATM   10  H3  UNL     1      -1.819  -1.044   1.176  1.00  0.00           H  
HETATM   11  H4  UNL     1       0.206   0.171   1.421  1.00  0.00           H  
HETATM   12  H5  UNL     1      -2.075   1.023  -0.419  1.00  0.00           H  
HETATM   13  H6  UNL     1      -1.686   1.547   1.276  1.00  0.00           H  
HETATM   14  H7  UNL     1      -0.784   2.225  -0.156  1.00  0.00           H  
HETATM   15  H8  UNL     1       1.323   1.255  -0.483  1.00  0.00           H  
HETATM   16  H9  UNL     1       0.187   0.424  -1.660  1.00  0.00           H  
HETATM   17  H10 UNL     1       3.078   0.101   0.525  1.00  0.00           H  
CONECT    1    2    8    9   10
CONECT    2    3    4   11
CONECT    3   12   13   14
CONECT    4    5   15   16
CONECT    5    6    6    7
CONECT    7   17
END
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SMILES for HMDB0000718 (Isovaleric acid)

CC(C)CC(O)=O
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INCHI for HMDB0000718 (Isovaleric acid)

InChI=1S/C5H10O2/c1-4(2)3-5(6)7/h4H,3H2,1-2H3,(H,6,7)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
3-Methyl-N-butyric acidChEBI
3-Methylbutanoic acidChEBI
3-MethylbuttersaeureChEBI
3-Methylbutyric acidChEBI
beta-Methylbutyric acidChEBI
Delphinic acidChEBI
Isobutylformic acidChEBI
Isopentanoic acidChEBI
Isopropylacetic acidChEBI
IsovalerateChEBI
Isovalerianic acidChEBI
IsovaleriansaeureChEBI
3-Methyl-N-butyrateGenerator
3-MethylbutanoateGenerator
3-MethylbutyrateGenerator
b-MethylbutyrateGenerator
b-Methylbutyric acidGenerator
beta-MethylbutyrateGenerator
Β-methylbutyrateGenerator
Β-methylbutyric acidGenerator
DelphinateGenerator
IsobutylformateGenerator
IsopentanoateGenerator
IsopropylacetateGenerator
IsovalerianateGenerator
3-Methyl butyric acidHMDB
3-Methylbutyric acid: isopropyl-acetateHMDB
3-Methylbutyric acid: isopropyl-acetic acidHMDB
3-Methyl-1-butanoic acidHMDB
Isovaleric acidKEGG
Chemical FormulaC5H10O2
Average Molecular Weight102.1317
Monoisotopic Molecular Weight102.068079564
IUPAC Name3-methylbutanoic acid
Traditional Nameisovaleric acid
CAS Registry Number503-74-2
SMILES
CC(C)CC(O)=O
InChI Identifier
InChI=1S/C5H10O2/c1-4(2)3-5(6)7/h4H,3H2,1-2H3,(H,6,7)
InChI KeyGWYFCOCPABKNJV-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentMethyl-branched fatty acids
Alternative Parents
Substituents
  • Methyl-branched fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Not AvailableNot Available
Physical Properties
StateLiquid
Experimental Molecular Properties
PropertyValueReference
Melting Point-29.3 °CNot Available
Boiling Point175.00 to 177.00 °C. @ 760.00 mm HgThe Good Scents Company Information System
Water Solubility40.7 mg/mLNot Available
LogP1.16HANSCH,C ET AL. (1995)
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility64.9 g/LALOGPS
logP1.26ALOGPS
logP1.21ChemAxon
logS-0.2ALOGPS
pKa (Strongest Acidic)5.01ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area37.3 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity26.42 m³·mol⁻¹ChemAxon
Polarizability10.99 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+120.95731661259
DarkChem[M-H]-116.77431661259
AllCCS[M+H]+124.82532859911
AllCCS[M-H]-126.72732859911
DeepCCS[M+H]+134.63930932474
DeepCCS[M-H]-131.87630932474
DeepCCS[M-2H]-168.24730932474
DeepCCS[M+Na]+143.14730932474
AllCCS[M+H]+124.832859911
AllCCS[M+H-H2O]+120.532859911
AllCCS[M+NH4]+128.832859911
AllCCS[M+Na]+130.032859911
AllCCS[M-H]-126.732859911
AllCCS[M+Na-2H]-130.732859911
AllCCS[M+HCOO]-135.032859911

Predicted Retention Times

Underivatized

Chromatographic MethodRetention TimeReference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.2.6 minutes32390414
Predicted by Siyang on May 30, 202211.3679 minutes33406817
Predicted by Siyang using ReTip algorithm on June 8, 20224.24 minutes32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid55.9 seconds40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid1422.8 seconds40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid395.5 seconds40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid125.9 seconds40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid249.3 seconds40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid89.5 seconds40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid410.7 seconds40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid458.5 seconds40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)122.1 seconds40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid804.7 seconds40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid333.2 seconds40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid1077.9 seconds40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid276.9 seconds40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid309.4 seconds40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate469.3 seconds40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA343.0 seconds40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water116.6 seconds40023050

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Isovaleric acidCC(C)CC(O)=O1653.4Standard polar33892256
Isovaleric acidCC(C)CC(O)=O818.2Standard non polar33892256
Isovaleric acidCC(C)CC(O)=O851.0Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Isovaleric acid,1TMS,isomer #1CC(C)CC(=O)O[Si](C)(C)C939.1Semi standard non polar33892256
Isovaleric acid,1TBDMS,isomer #1CC(C)CC(=O)O[Si](C)(C)C(C)(C)C1150.3Semi standard non polar33892256
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.6 (0.3-2.7) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified4.896 uMChildren (1 - 13 years old)FemaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.35 (0-2.7) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 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 and Quantified0-93506.717 nmol/g wet fecesChildren (1-13 years old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not AvailableNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not Specified
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 SpecifiedNormal 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 and Quantified70-3230 nmol/g wet fecesAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified3200 (800-5900) nmol/g wet fecesAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
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 and Quantified1280 +/- 920 nmol/g wet fecesAdult (>18 years old)Female
Normal		 details
FecesDetected and Quantified1622.807 +/- 672.465 nmol/g wet fecesNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified54.03 +/- 39.40 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
 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
UrineDetected and Quantified0.07 (0.0-0.13) umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
 details
UrineDetected and Quantified<2.214 umol/mmol creatinineChildren (1 - 13 years old)FemaleNormal details
UrineDetected and Quantified3.415 +/- 0.858 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected and Quantified3.5 (1.1-6.7) umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
 details
UrineDetected and Quantified0.42 ± 0.02 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified1.4 (0.8-2.5) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified41.0 +/- 25.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified75.393 uMChildren (1 - 13 years old)FemaleIsovaleric acidemia details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Clostridium difficile infection		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specifiedsymptomatic uncomplicated diverticular disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specifiedasymptomatic diverticulosis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Crohn's disease 		details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis		 details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothPervasive Developmental Disorder Not Otherwise Specified details
FecesDetected and Quantified0.000666 (0.000069-0.00578) nmol/g wet fecesNot SpecifiedNot Specified
Irritable bowel syndrome		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Campylobacter jejuni infection		 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 QuantifiedAdult (>18 years old)Both
Ulcerative colitis		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Nonalcoholic fatty liver disease (NAFLD)		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected and Quantified0-173762.570 nmol/g wet fecesChildren (1-13 years old)Not Specified
Treated celiac disease		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Crohn's disease		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Ulcerative colitis		 details
FecesDetected and Quantified0.00138 (0.000676-0.00398) nmol/g wet fecesNot SpecifiedNot Specified
Crohns disease		 details
FecesDetected and Quantified0.000274 (0.0000098-0.00942) nmol/g wet fecesNot SpecifiedNot Specified
Ulcerative colitis		 details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)Bothautism details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleAttachment loss  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MalePeriodontal Probing Depth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleSupragingival Plaque details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleTooth Decay details
UrineDetected and Quantified2.559 +/- 2.305 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected and Quantified14.391 umol/mmol creatinineChildren (1 - 13 years old)FemaleIsovaleric acidemia details
Associated Disorders and Diseases
Disease References
Isovaleric acidemia
  1. Krieger I, Tanaka K: Therapeutic effects of glycine in isovaleric acidemia. Pediatr Res. 1976 Jan;10(1):25-9. [PubMed:1246461 ]
Ulcerative colitis
  1. Garner CE, Smith S, de Lacy Costello B, White P, Spencer R, Probert CS, Ratcliffe NM: Volatile organic compounds from feces and their potential for diagnosis of gastrointestinal disease. FASEB J. 2007 Jun;21(8):1675-88. Epub 2007 Feb 21. [PubMed:17314143 ]
  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. Walton C, Fowler DP, Turner C, Jia W, Whitehead RN, Griffiths L, Dawson C, Waring RH, Ramsden DB, Cole JA, Cauchi M, Bessant C, Hunter JO: Analysis of volatile organic compounds of bacterial origin in chronic gastrointestinal diseases. Inflamm Bowel Dis. 2013 Sep;19(10):2069-78. doi: 10.1097/MIB.0b013e31829a91f6. [PubMed:23867873 ]
  4. De Preter V, Machiels K, Joossens M, Arijs I, Matthys C, Vermeire S, Rutgeerts P, Verbeke K: Faecal metabolite profiling identifies medium-chain fatty acids as discriminating compounds in IBD. Gut. 2015 Mar;64(3):447-58. doi: 10.1136/gutjnl-2013-306423. Epub 2014 May 8. [PubMed:24811995 ]
  5. Ahmed I, Greenwood R, Costello B, Ratcliffe N, Probert CS: Investigation of faecal volatile organic metabolites as novel diagnostic biomarkers in inflammatory bowel disease. Aliment Pharmacol Ther. 2016 Mar;43(5):596-611. doi: 10.1111/apt.13522. Epub 2016 Jan 25. [PubMed:26806034 ]
Irritable bowel syndrome
  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. Walton C, Fowler DP, Turner C, Jia W, Whitehead RN, Griffiths L, Dawson C, Waring RH, Ramsden DB, Cole JA, Cauchi M, Bessant C, Hunter JO: Analysis of volatile organic compounds of bacterial origin in chronic gastrointestinal diseases. Inflamm Bowel Dis. 2013 Sep;19(10):2069-78. doi: 10.1097/MIB.0b013e31829a91f6. [PubMed:23867873 ]
Nonalcoholic fatty liver disease
  1. Raman M, Ahmed I, Gillevet PM, Probert CS, Ratcliffe NM, Smith S, Greenwood R, Sikaroodi M, Lam V, Crotty P, Bailey J, Myers RP, Rioux KP: Fecal microbiome and volatile organic compound metabolome in obese humans with nonalcoholic fatty liver disease. Clin Gastroenterol Hepatol. 2013 Jul;11(7):868-75.e1-3. doi: 10.1016/j.cgh.2013.02.015. Epub 2013 Feb 27. [PubMed:23454028 ]
Colorectal cancer
  1. 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 ]
  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. 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 ]
  4. 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 ]
  5. Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20. [PubMed:28587349 ]
Celiac disease
  1. Di Cagno R, De Angelis M, De Pasquale I, Ndagijimana M, Vernocchi P, Ricciuti P, Gagliardi F, Laghi L, Crecchio C, Guerzoni ME, Gobbetti M, Francavilla R: Duodenal and faecal microbiota of celiac children: molecular, phenotype and metabolome characterization. BMC Microbiol. 2011 Oct 4;11:219. doi: 10.1186/1471-2180-11-219. [PubMed:21970810 ]
Crohn's disease
  1. Walton C, Fowler DP, Turner C, Jia W, Whitehead RN, Griffiths L, Dawson C, Waring RH, Ramsden DB, Cole JA, Cauchi M, Bessant C, Hunter JO: Analysis of volatile organic compounds of bacterial origin in chronic gastrointestinal diseases. Inflamm Bowel Dis. 2013 Sep;19(10):2069-78. doi: 10.1097/MIB.0b013e31829a91f6. [PubMed:23867873 ]
  2. De Preter V, Machiels K, Joossens M, Arijs I, Matthys C, Vermeire S, Rutgeerts P, Verbeke K: Faecal metabolite profiling identifies medium-chain fatty acids as discriminating compounds in IBD. Gut. 2015 Mar;64(3):447-58. doi: 10.1136/gutjnl-2013-306423. Epub 2014 May 8. [PubMed:24811995 ]
  3. Ahmed I, Greenwood R, Costello B, Ratcliffe N, Probert CS: Investigation of faecal volatile organic metabolites as novel diagnostic biomarkers in inflammatory bowel disease. Aliment Pharmacol Ther. 2016 Mar;43(5):596-611. doi: 10.1111/apt.13522. Epub 2016 Jan 25. [PubMed:26806034 ]
Autism
  1. 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 ]
Pervasive developmental disorder not otherwise specified
  1. 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
  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 ]
Attachment loss
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Supragingival Plaque
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Tooth Decay
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
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
DrugBank IDDB03750
Phenol Explorer Compound IDNot Available
FooDB IDFDB001324
KNApSAcK IDC00001189
Chemspider ID10001
KEGG Compound IDC08262
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia Link3-Methylbutanoic acid
METLIN ID109
PubChem Compound10430
PDB IDNot Available
ChEBI ID28484
Food Biomarker OntologyNot Available
VMH IDISOVAL
MarkerDB IDMDB00000234
Good Scents IDrw1056411
References
Synthesis ReferenceImada, Yukio; Mizuno, Sumiko; Mikawa, Takashi. Manufacture of isovaleric acid and 2-methylbutyric acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Silva MF, Selhorst J, Overmars H, van Gennip AH, Maya M, Wanders RJ, de Almeida IT, Duran M: Characterization of plasma acylcarnitines in patients under valproate monotherapy using ESI-MS/MS. Clin Biochem. 2001 Nov;34(8):635-8. [PubMed:11849623 ]
  2. Hoffmann GF, von Kries R, Klose D, Lindner M, Schulze A, Muntau AC, Roschinger W, Liebl B, Mayatepek E, Roscher AA: Frequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany. Eur J Pediatr. 2004 Feb;163(2):76-80. Epub 2004 Jan 9. [PubMed:14714182 ]
  3. Arthur K, Hommes FA: Simple isotope dilution assay for propionic acid and isovaleric acid. J Chromatogr B Biomed Appl. 1995 Nov 3;673(1):132-5. [PubMed:8925066 ]
  4. Ara K, Hama M, Akiba S, Koike K, Okisaka K, Hagura T, Kamiya T, Tomita F: Foot odor due to microbial metabolism and its control. Can J Microbiol. 2006 Apr;52(4):357-64. [PubMed:16699586 ]
  5. Ackman RG: Birthweights in the Faroe Islands: possible role of isovaleric acid. J Intern Med. 1989 Feb;225(2):73-5. [PubMed:2646392 ]

Enzymes

Enzyme Details
1. UDP-glucuronosyltransferase 1-1
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
Reactions
Isovaleric acid → 3,4,5-trihydroxy-6-[(3-methylbutanoyl)oxy]oxane-2-carboxylic aciddetails