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Record Information |
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Version | 5.0 |
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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 | 2022-09-22 18:35:06 UTC |
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HMDB ID | HMDB0000665 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | Leucinic acid |
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Description | Leucinic acid, also known as leucic acid, 2-hydroxyisocaproic acid or 2-hydroxy-4-methylvaleric acid, belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group. Leucinic acid is a valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position. It is an alpha-hydroxy analogue of leucine and a metabolite of the branched-chain amino acid leucine. Leucinic acid is found in all organisms ranging from bacteria to plants to animals. Leucinic acid has been found in a patient with dihydrolipoyl dehydrogenase (DLD) deficiency (PMID: 6688766 ). DLD deficiency is caused by mutations in the DLD gene and is inherited in an autosomal recessive manner. A common feature of dihydrolipoamide dehydrogenase deficiency is a potentially life-threatening buildup of lactic acid in tissues (lactic acidosis), which can cause nausea, vomiting, severe breathing problems, and an abnormal heartbeat. Neurological problems are also common in this condition; the first symptoms in affected infants are often decreased muscle tone (hypotonia) and extreme tiredness (lethargy). As the problems worsen, affected infants can have difficulty feeding, decreased alertness, and seizures. Liver problems can also occur in dihydrolipoamide dehydrogenase deficiency, ranging from an enlarged liver (hepatomegaly) to life-threatening liver failure. In some affected people, liver disease, which can begin anytime from infancy to adulthood, is the primary symptom. Leucinic acid is also present in the urine of patients with short bowel syndrome (PMID: 4018104 ) |
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Structure | InChI=1S/C6H12O3/c1-4(2)3-5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9) |
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Synonyms | Value | Source |
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2-Hydroxyisocaproic acid | ChEBI | 2-Hydroxyisohexanoic acid | ChEBI | alpha-Hydroxyisocaproic acid | ChEBI | Leucic acid | ChEBI | 2-Hydroxyisocaproate | Generator | 2-Hydroxyisohexanoate | Generator | a-Hydroxyisocaproate | Generator | a-Hydroxyisocaproic acid | Generator | alpha-Hydroxyisocaproate | Generator | Α-hydroxyisocaproate | Generator | Α-hydroxyisocaproic acid | Generator | Leucate | Generator | Leucinate | Generator | alpha-Hydroxyisocaproic acid, (R)-isomer | HMDB | alpha-Hydroxyisocaproic acid, calcium (2:1) salt, (S)-isomer | HMDB | alpha-Hydroxyisocaproic acid, monosodium salt | HMDB | alpha-Hydroxyisocaproic acid, (S)-isomer | HMDB | 2-Hydroxy-4-methyl-valerate | HMDB | 2-Hydroxy-4-methyl-valeric acid | HMDB | 2-Hydroxy-4-methylpentanoate | HMDB | 2-Hydroxy-4-methylpentanoic acid | HMDB | 2-Hydroxy-4-methylvalerate | HMDB | 2-Hydroxy-4-methylvaleric acid | HMDB | a-Hydroxy-iso-caproate | HMDB | a-Hydroxy-iso-caproic acid | HMDB | alpha-Hydroxy-iso-caproate | HMDB | alpha-Hydroxy-iso-caproic acid | HMDB | DL-2-Hydroxy-4-methylpentanoate | HMDB | DL-2-Hydroxy-4-methylpentanoic acid | HMDB | DL-2-Hydroxyisocaproate | HMDB | DL-2-Hydroxyisocaproic acid | HMDB | DL-a-Hydroxyisocaproate | HMDB | DL-a-Hydroxyisocaproic acid | HMDB | DL-alpha-Hydroxyisocaproate | HMDB | DL-alpha-Hydroxyisocaproic acid | HMDB | DL-Leucate | HMDB | DL-Leucic acid | HMDB | Leucinic acid | ChEBI |
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Chemical Formula | C6H12O3 |
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Average Molecular Weight | 132.1577 |
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Monoisotopic Molecular Weight | 132.07864425 |
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IUPAC Name | 2-hydroxy-4-methylpentanoic acid |
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Traditional Name | leucate |
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CAS Registry Number | 498-36-2 |
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SMILES | CC(C)CC(O)C(O)=O |
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InChI Identifier | InChI=1S/C6H12O3/c1-4(2)3-5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9) |
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InChI Key | LVRFTAZAXQPQHI-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | Belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group. |
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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 | Hydroxy fatty acids |
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Alternative Parents | |
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Substituents | - Branched fatty acid
- Hydroxy fatty acid
- Methyl-branched fatty acid
- Alpha-hydroxy acid
- Hydroxy acid
- Secondary alcohol
- Monocarboxylic acid or derivatives
- Carboxylic acid
- Carboxylic acid derivative
- Organooxygen compound
- Carbonyl group
- Alcohol
- Hydrocarbon derivative
- Organic oxide
- Organic oxygen compound
- 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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Physiological effect | Not Available |
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Disposition | |
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Process | |
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Role | |
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Physical Properties |
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State | Solid |
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Experimental Molecular Properties | |
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Experimental Chromatographic Properties | Not Available |
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Predicted Molecular Properties | |
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Predicted Chromatographic Properties | Predicted Collision Cross SectionsPredicted Kovats Retention IndicesUnderivatizedDerivatizedDerivative Name / Structure | SMILES | Kovats RI Value | Column Type | Reference |
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Leucinic acid,1TMS,isomer #1 | CC(C)CC(O[Si](C)(C)C)C(=O)O | 1197.3 | Semi standard non polar | 33892256 | Leucinic acid,1TMS,isomer #2 | CC(C)CC(O)C(=O)O[Si](C)(C)C | 1129.2 | Semi standard non polar | 33892256 | Leucinic acid,2TMS,isomer #1 | CC(C)CC(O[Si](C)(C)C)C(=O)O[Si](C)(C)C | 1243.6 | Semi standard non polar | 33892256 | Leucinic acid,1TBDMS,isomer #1 | CC(C)CC(O[Si](C)(C)C(C)(C)C)C(=O)O | 1403.1 | Semi standard non polar | 33892256 | Leucinic acid,1TBDMS,isomer #2 | CC(C)CC(O)C(=O)O[Si](C)(C)C(C)(C)C | 1357.2 | Semi standard non polar | 33892256 | Leucinic acid,2TBDMS,isomer #1 | CC(C)CC(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C | 1653.2 | Semi standard non polar | 33892256 |
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Spectra |
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| GC-MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (Non-derivatized) - 70eV, Positive | splash10-0006-9000000000-0883a44db52a6c25fe99 | 2016-09-22 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (2 TMS) - 70eV, Positive | splash10-02gc-9440000000-7fffd162011678b17f4a | 2017-10-06 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (TMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (TMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (TBDMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (TBDMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Leucinic acid GC-MS (TBDMS_2_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum |
MS/MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid Quattro_QQQ 10V, Positive-QTOF (Annotated) | splash10-014r-9000000000-5e0d546d1d0e6bb8c48c | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid Quattro_QQQ 25V, Positive-QTOF (Annotated) | splash10-014i-9100000000-ed29dea8a5c4015ec5f9 | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid Quattro_QQQ 40V, Positive-QTOF (Annotated) | splash10-0a4i-8900000000-44e1b9618ac09f8f20b8 | 2012-07-24 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF | splash10-001r-7900000000-7ab0c9dc80e61fa580c9 | 2012-08-31 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid LC-ESI-QTOF , negative-QTOF | splash10-001r-7900000000-7ab0c9dc80e61fa580c9 | 2017-09-14 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid 35V, Negative-QTOF | splash10-000i-9100000000-b13176bdf03a9a3860aa | 2021-09-20 | HMDB team, MONA | View Spectrum | Experimental LC-MS/MS | LC-MS/MS Spectrum - Leucinic acid 35V, Positive-QTOF | splash10-0006-9000000000-702157a92667487bd5f6 | 2021-09-20 | HMDB team, MONA | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 10V, Positive-QTOF | splash10-00lr-7900000000-d13b28975eefc974bbca | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 20V, Positive-QTOF | splash10-0ap0-9200000000-aaa07e44b97e9617d7f1 | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 40V, Positive-QTOF | splash10-0a4i-9000000000-931176b5fcc7e8bde29c | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 10V, Negative-QTOF | splash10-001i-4900000000-c818e3e6474333b25326 | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 20V, Negative-QTOF | splash10-053i-9200000000-bd611de2f2748fe8a7fa | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 40V, Negative-QTOF | splash10-0ap0-9000000000-4e37e159ea3117d8a516 | 2016-09-12 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 10V, Negative-QTOF | splash10-0019-9700000000-a7d0b1ce1c211b31bc0d | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 20V, Negative-QTOF | splash10-00kr-9100000000-504826b66e199a65ef09 | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 40V, Negative-QTOF | splash10-014l-9000000000-ccecb4a470c359949fec | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 10V, Positive-QTOF | splash10-014i-9000000000-3019a9d0620e7da01963 | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 20V, Positive-QTOF | splash10-00kf-9000000000-e587d44396e53f2409fa | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Leucinic acid 40V, Positive-QTOF | splash10-0006-9000000000-457ece1d510dad645c89 | 2021-09-25 | Wishart Lab | View Spectrum |
NMR SpectraSpectrum Type | Description | Deposition Date | Source | View |
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Experimental 1D NMR | 1H NMR Spectrum (1D, 500 MHz, H2O, experimental) | 2012-12-04 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 100 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 100 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 1000 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 1000 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 200 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 200 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 300 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 300 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 400 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 400 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 500 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 500 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 600 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 600 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 700 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 700 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 800 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 800 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 1H NMR Spectrum (1D, 900 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 900 MHz, D2O, predicted) | 2021-09-29 | Wishart Lab | View Spectrum | Experimental 2D NMR | [1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental) | 2012-12-05 | Wishart Lab | View Spectrum |
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Biological Properties |
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Cellular Locations | |
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Biospecimen Locations | |
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Tissue Locations | Not Available |
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Pathways | |
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Normal Concentrations |
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Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0-50 umol/mmol creatinine | Newborn (0-30 days old) | Both | Normal | | details | Urine | Detected and Quantified | 0.0 umol/mmol creatinine | Children (1 - 18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0-0.39 umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Blood | Expected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Asthma | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Colorectal cancer | | details | Feces | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Colorectal cancer | | details |
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Associated Disorders and Diseases |
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Disease References | Colorectal cancer |
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- 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 ]
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Associated OMIM IDs | |
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | FDB022171 |
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KNApSAcK ID | Not Available |
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Chemspider ID | 83753 |
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KEGG Compound ID | Not Available |
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BioCyc ID | Not Available |
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BiGG ID | Not Available |
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Wikipedia Link | Not Available |
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METLIN ID | 5635 |
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PubChem Compound | 92779 |
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PDB ID | Not Available |
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ChEBI ID | 59783 |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | Not Available |
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Good Scents ID | rw1488981 |
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References |
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Synthesis Reference | Camien, Merill N.; Fowler, Audree V.; Dunn, Max S. Production of a-hydroxy fatty acids by two strains of Lactobacillus casei. Archives of Biochemistry and Biophysics (1959), 83 408-18. |
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Material Safety Data Sheet (MSDS) | Download (PDF) |
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General References | - 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 ]
- Jakobs C, Sweetman L, Nyhan WL: Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clin Chim Acta. 1984 Jul 16;140(2):157-66. [PubMed:6467607 ]
- Spaapen LJ, Ketting D, Wadman SK, Bruinvis L, Duran M: Urinary D-4-hydroxyphenyllactate, D-phenyllactate and D-2-hydroxyisocaproate, abnormalities of bacterial origin. J Inherit Metab Dis. 1987;10(4):383-90. [PubMed:3126358 ]
- Haan E, Brown G, Bankier A, Mitchell D, Hunt S, Blakey J, Barnes G: Severe illness caused by the products of bacterial metabolism in a child with a short gut. Eur J Pediatr. 1985 May;144(1):63-5. [PubMed:4018104 ]
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