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| Record Information |
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| Version | 4.0 |
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| Status | Detected and Quantified |
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| Creation Date | 2005-11-16 15:48:42 UTC |
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| Update Date | 2018-04-16 17:02:09 UTC |
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| HMDB ID | HMDB0000718 |
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| Secondary Accession Numbers | |
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| Metabolite Identification |
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| Common Name | Isovaleric acid |
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| Description | Isovaleric 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. |
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| Structure | |
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| Synonyms | | Value | Source |
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| 3-Methyl-N-butyric acid | ChEBI | | 3-Methylbutanoic acid | ChEBI | | 3-Methylbuttersaeure | ChEBI | | 3-Methylbutyric acid | ChEBI | | beta-Methylbutyric acid | ChEBI | | Delphinic acid | ChEBI | | Isobutylformic acid | ChEBI | | Isopentanoic acid | ChEBI | | Isopropylacetic acid | ChEBI | | Isovalerate | ChEBI | | Isovalerianic acid | ChEBI | | Isovaleriansaeure | ChEBI | | 3-Methyl-N-butyrate | Generator | | 3-Methylbutanoate | Generator | | 3-Methylbutyrate | Generator | | b-Methylbutyrate | Generator | | b-Methylbutyric acid | Generator | | beta-Methylbutyrate | Generator | | β-methylbutyrate | Generator | | β-methylbutyric acid | Generator | | Delphinate | Generator | | Isobutylformate | Generator | | Isopentanoate | Generator | | Isopropylacetate | Generator | | Isovalerianate | Generator | | 3-Methyl butyric acid | HMDB | | 3-Methylbutyric acid: isopropyl-acetate | HMDB | | 3-Methylbutyric acid: isopropyl-acetic acid | HMDB |
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| Chemical Formula | C5H10O2 |
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| Average Molecular Weight | 102.1317 |
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| Monoisotopic Molecular Weight | 102.068079564 |
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| IUPAC Name | 3-methylbutanoic acid |
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| Traditional Name | isovaleric acid |
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| CAS Registry Number | 503-74-2 |
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| SMILES | CC(C)CC(O)=O |
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| InChI Identifier | InChI=1S/C5H10O2/c1-4(2)3-5(6)7/h4H,3H2,1-2H3,(H,6,7) |
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| InChI Key | GWYFCOCPABKNJV-UHFFFAOYSA-N |
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| Chemical Taxonomy |
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| Description | This compound 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. |
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| Kingdom | Organic compounds |
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| Super Class | Lipids and lipid-like molecules |
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| Class | Fatty Acyls |
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| Sub Class | Fatty acids and conjugates |
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| Direct Parent | Methyl-branched fatty acids |
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| Alternative Parents | |
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| 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
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| Molecular Framework | Aliphatic acyclic compounds |
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| External Descriptors | |
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| Ontology |
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| Disposition | Route of exposure: Source: Biological location: |
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| Process | Naturally occurring process: |
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| Role | Biological role: Industrial application: |
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| Physical Properties |
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| State | Liquid |
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| Experimental Properties | | Property | Value | Reference |
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| Melting Point | -29.3 °C | Not Available | | Boiling Point | Not Available | Not Available | | Water Solubility | 40.7 mg/mL | Not Available | | LogP | 1.16 | HANSCH,C ET AL. (1995) |
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| Predicted Properties | |
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| Spectra |
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| Spectra | | Spectrum Type | Description | Splash Key | |
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| GC-MS | GC-MS Spectrum - EI-B (Non-derivatized) | splash10-03dl-9000000000-9e19eb5f5137b78591df | View in MoNA |
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| GC-MS | GC-MS Spectrum - EI-B (Non-derivatized) | splash10-03dl-9000000000-43967f1315b6b575f330 | View in MoNA |
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| Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-052f-9000000000-2825512cee017b3ab87c | View in MoNA |
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| Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive | splash10-059f-9200000000-628e11f1ffbaa913ef57 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated) | splash10-0gvo-9300000000-16b5d15d26810b72bb36 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated) | splash10-0006-9000000000-692791435c37fa74a692 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated) | splash10-0006-9000000000-b4c1b59653cd4d803637 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - EI-B (HITACHI RMU-7M) , Positive | splash10-03dl-9000000000-b0f7e399edb7f95572ed | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - EI-B (HITACHI M-80B) , Positive | splash10-03dl-9000000000-c8be68873f336c8de6d6 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative | splash10-0udi-0900000000-d0f20bc9813dcba79e8b | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative | splash10-0udi-0900000000-63b09019c280c88ee64d | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative | splash10-0udi-1900000000-ef9ee29c1febd59e6a00 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0udi-0900000000-d0f20bc9813dcba79e8b | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0udi-0900000000-63b09019c280c88ee64d | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , negative | splash10-0udi-1900000000-ef9ee29c1febd59e6a00 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-000i-9200000000-e825f0aa9ab54c1695d3 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-0a4l-9000000000-9ba126f982634fdc9406 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-052f-9000000000-afb863a56c8763c30251 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-0zfr-8900000000-244535e4070d536a29b3 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-0pc0-9300000000-93ed2bc326380cb0d880 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-0a4l-9000000000-44b2fa11a5a6daab8dd0 | View in MoNA |
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| MS | Mass Spectrum (Electron Ionization) | splash10-03dl-9000000000-e7c77b2bc7f3ac5191e9 | View in MoNA |
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| 1D NMR | 13C NMR Spectrum | Not Available | View in JSpectraViewer |
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| 1D NMR | 1H NMR Spectrum | Not Available | View in JSpectraViewer |
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| 1D NMR | 1H NMR Spectrum | Not Available | View in JSpectraViewer |
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| 1D NMR | 13C NMR Spectrum | Not Available | View in JSpectraViewer |
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| 1D NMR | 1H NMR Spectrum | Not Available | View in JSpectraViewer |
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| 1D NMR | 13C NMR Spectrum | Not Available | View in JSpectraViewer |
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| 2D NMR | [1H,13C] 2D NMR Spectrum | Not Available | View in JSpectraViewer |
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| Biological Properties |
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| Cellular Locations | |
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| Biospecimen Locations | - Blood
- Cerebrospinal Fluid (CSF)
- Feces
- Saliva
- Urine
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| Tissue Location | Not Available |
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| Pathways | | Name | SMPDB/Pathwhiz | KEGG | Not Available
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| Normal Concentrations |
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| Blood | Detected and Quantified | 1.6 (0.3-2.7) uM | Adult (>18 years old) | Both | Normal | | details | | Blood | Detected and Quantified | 4.896 uM | Children (1 - 13 years old) | Female | Normal | | details | | Cerebrospinal Fluid (CSF) | Detected and Quantified | 1.35 (0-2.7) uM | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Infant (0-1 year old) | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Infant (0-1 year old) | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Children (1-13 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Not Specified | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Infant (0-1 year old) | Not Available | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Female | Normal | | details | | Feces | Detected and Quantified | 1622.807 +/- 672.465 nmol/g wet feces | Not Specified | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected and Quantified | 1280 +/- 920 nmol/g wet feces | Adult (>18 years old) | Female | Normal | | details | | Feces | Detected and Quantified | 0-93506.717 nmol/g wet feces | Children (1-13 years old) | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Not Specified | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected and Quantified | 70-3230 nmol/g wet feces | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Normal | | details | | Saliva | Detected and Quantified | 54.03 +/- 39.40 uM | Adult (>18 years old) | Both | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Saliva | Detected but not Quantified | | Adult (>18 years old) | Male | Normal | | details | | Urine | Detected and Quantified | 3.415 +/- 0.858 umol/mmol creatinine | Children (1 - 13 years old) | Not Specified | Normal | | details | | Urine | Detected and Quantified | 1.4 (0.8-2.5) umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details | | Urine | Detected and Quantified | 41.0 +/- 25.0 umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details | | Urine | Detected and Quantified | 0.42 ± 0.02 umol/mmol creatinine | Adult (>18 years old) | Male | Normal | | details | | Urine | Detected and Quantified | 0.07 (0.0-0.13) umol/mmol creatinine | Children (1-13 years old) | Both | Normal | | details | | Urine | Detected and Quantified | 3.5 (1.1-6.7) umol/mmol creatinine | Infant (0-1 year old) | Both | Normal | | details | | Urine | Detected and Quantified | <2.214 umol/mmol creatinine | Children (1 - 13 years old) | Female | Normal | | details |
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| Abnormal Concentrations |
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| Blood | Detected and Quantified | 75.393 uM | Children (1 - 13 years old) | Female | Isovaleric acidemia | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal cancer | | details | | Feces | Detected but not Quantified | | Children (1-13 years old) | Both | autism | | details | | Feces | Detected but not Quantified | | Children (1-13 years old) | Both | Pervasive Developmental Disorder Not Otherwise Specified | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Irritable bowel syndrome | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Ulcerative colitis | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Crohns disease | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Ulcerative colitis | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Ulcerative colitis | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Crohn's disease | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal Cancer | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal Cancer | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Female | Myalgic encephalomyelitis/chronic fatigue syndrome | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal Cancer | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal Cancer | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal cancer | | details | | Feces | Detected but not Quantified | | Not Specified | Not Specified | Crohn's disease | | details | | Feces | Detected but not Quantified | | Not Specified | Not Specified | Ulcerative colitis | | details | | Feces | Detected and Quantified | 0.00138 (0.000676-0.00398) nmol/g wet feces | Not Specified | Not Specified | Crohns disease | | details | | Feces | Detected and Quantified | 0.000274 (0.0000098-0.00942) nmol/g wet feces | Not Specified | Not Specified | Ulcerative colitis | | details | | Feces | Detected and Quantified | 0.000666 (0.000069-0.00578) nmol/g wet feces | Not Specified | Not Specified | Irritable bowel syndrome | | details | | Feces | Detected and Quantified | 0-173762.570 nmol/g wet feces | Children (1-13 years old) | Not Specified | Treated celiac disease | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Not Specified | asymptomatic diverticulosis | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Not Specified | symptomatic uncomplicated diverticular disease | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Campylobacter jejuni infection | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Clostridium difficile infection | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Ulcerative Colitis | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Colorectal cancer | | details | | Feces | Detected but not Quantified | | Adult (>18 years old) | Both | Nonalcoholic fatty liver disease (NAFLD) | | details | | Urine | Detected and Quantified | 2.559 +/- 2.305 umol/mmol creatinine | Children (1 - 13 years old) | Not Specified | Eosinophilic esophagitis | | details | | Urine | Detected and Quantified | 14.391 umol/mmol creatinine | Children (1 - 13 years old) | Female | Isovaleric acidemia | | details |
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| Associated Disorders and Diseases |
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| Disease References | | Celiac disease |
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- 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 |
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- 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 ]
| | Irritable bowel syndrome |
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- 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 ]
| | Isovaleric acidemia |
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- Krieger I, Tanaka K: Therapeutic effects of glycine in isovaleric acidemia. Pediatr Res. 1976 Jan;10(1):25-9. [PubMed:1246461 ]
| | Ulcerative colitis |
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- 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 ]
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| Associated OMIM IDs | |
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| External Links |
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| DrugBank ID | DB03750 |
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| Phenol Explorer Compound ID | Not Available |
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| FoodDB ID | FDB001324 |
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| KNApSAcK ID | C00001189 |
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| Chemspider ID | 10001 |
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| KEGG Compound ID | C08262 |
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| BioCyc ID | Not Available |
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| BiGG ID | Not Available |
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| Wikipedia Link | 3-Methylbutanoic acid |
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| METLIN ID | 109 |
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| PubChem Compound | 10430 |
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| PDB ID | IVA |
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| ChEBI ID | 28484 |
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| References |
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| Synthesis Reference | Imada, Yukio; Mizuno, Sumiko; Mikawa, Takashi. Manufacture of isovaleric acid and 2-methylbutyric acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp. |
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| Material Safety Data Sheet (MSDS) | Download (PDF) |
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| General References | - 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 ]
- 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 ]
- 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 ]
- 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 ]
- Ackman RG: Birthweights in the Faroe Islands: possible role of isovaleric acid. J Intern Med. 1989 Feb;225(2):73-5. [PubMed:2646392 ]
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