| Record Information |
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| Version | 3.6 |
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| Creation Date | 2005-11-16 15:48:42 UTC |
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| Update Date | 2017-03-02 21:26:06 UTC |
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| HMDB ID | HMDB00695 |
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| Secondary Accession Numbers | |
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| Metabolite Identification |
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| Common Name | Ketoleucine |
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| Description | Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain; this is of relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients. (PMID 14636955 ). |
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| Structure | |
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| Synonyms | | Value | Source |
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| 2-oxo-4-METHYLPENTANOIC ACID | ChEBI | | 2-Oxoisocaproate | ChEBI | | 4-Methyl-2-oxopentanoate | ChEBI | | 4-Methyl-2-oxovaleric acid | ChEBI | | alpha-Ketoisocaproic acid | ChEBI | | 2-oxo-4-METHYLPENTANOate | Generator | | 2-Oxoisocaproic acid | Generator | | 4-Methyl-2-oxopentanoic acid | Generator | | 4-Methyl-2-oxovalerate | Generator | | a-Ketoisocaproate | Generator | | a-Ketoisocaproic acid | Generator | | alpha-Ketoisocaproate | Generator | | α-ketoisocaproate | Generator | | α-ketoisocaproic acid | Generator | | 2-keto-4-Methylvalerate | HMDB | | 2-keto-4-Methylvaleric acid | HMDB | | 2-Ketoisocaproate | HMDB | | 2-Ketoisocaproic acid | HMDB | | 2-oxo-4-Methylvalerate | HMDB | | 2-oxo-4-Methylvaleric acid | HMDB | | 2-Oxoleucine | HMDB | | 4-Methyl-2-oxo-valerate | HMDB | | 4-Methyl-2-oxo-valeric acid | HMDB | | a-Ketoisocapronate | HMDB | | a-Ketoisocapronic acid | HMDB | | a-Oxoisocaproate | HMDB | | a-Oxoisocaproic acid | HMDB | | alpha-keto-Isocaproate | HMDB | | alpha-keto-Isocaproic acid | HMDB | | alpha-Ketoisocapronate | HMDB | | alpha-Ketoisocapronic acid | HMDB | | alpha-Oxoisocaproate | HMDB | | alpha-Oxoisocaproic acid | HMDB | | Ketoisocaproate | HMDB | | Ketoisocaproic acid | HMDB | | Methyloxovalerate | HMDB | | Methyloxovaleric acid | HMDB | | Oxoisocaproate | HMDB | | Oxoisocaproic acid | HMDB | | alpha-Ketoisocaproic acid, calcium salt | MeSH | | alpha-Ketoisocaproic acid, sodium salt | MeSH |
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| Chemical Formula | C6H10O3 |
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| Average Molecular Weight | 130.1418 |
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| Monoisotopic Molecular Weight | 130.062994186 |
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| IUPAC Name | 4-methyl-2-oxopentanoic acid |
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| Traditional Name | ketoisocaproate |
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| CAS Registry Number | 816-66-0 |
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| SMILES | CC(C)CC(=O)C(O)=O |
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| InChI Identifier | InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9) |
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| InChI Key | BKAJNAXTPSGJCU-UHFFFAOYSA-N |
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| Chemical Taxonomy |
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| Description | This compound belongs to the class of chemical entities known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms. |
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| Kingdom | Chemical entities |
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| Super Class | Organic compounds |
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| Class | Organic acids and derivatives |
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| Sub Class | Keto acids and derivatives |
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| Direct Parent | Short-chain keto acids and derivatives |
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| Alternative Parents | |
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| Substituents | - Branched fatty acid
- Methyl-branched fatty acid
- Short-chain keto acid
- Alpha-keto acid
- Fatty acyl
- Alpha-hydroxy ketone
- Ketone
- Carboxylic acid derivative
- Carboxylic acid
- Monocarboxylic acid or derivatives
- Carbonyl group
- Hydrocarbon derivative
- Organic oxygen compound
- Organooxygen compound
- Organic oxide
- 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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| Status | Detected and Quantified |
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| Origin | |
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| Biofunction | - Cell signaling
- Component of Pantothenate and CoA biosynthesis
- Component of Valine, leucine and isoleucine biosynthesis
- Fuel and energy storage
- Fuel or energy source
- Membrane integrity/stability
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| Application | - Nutrients
- Stabilizers
- Surfactants and Emulsifiers
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| Cellular locations | - Cytoplasm
- Extracellular
- Membrane
- Mitochondria
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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 | 8 - 10 °C | Not Available | | Boiling Point | Not Available | Not Available | | Water Solubility | 32 mg/mL | Not Available | | LogP | Not Available | Not Available |
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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 - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS) | splash10-000i-9400000000-49d055f04bfe1877ac5b | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS) | splash10-029j-8930000000-c6c54bf6ee2995a2195b | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) | splash10-0002-1910000000-0ba38257e25873312809 | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-MS (2 TMS) | splash10-0a5c-4930000000-61247369a805fa2a77a7 | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-MS (1 MEOX; 1 TMS) | splash10-000i-9510000000-8bfc5a54e29f65edf361 | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-MS (1 MEOX; 1 TMS) | splash10-00lj-9620000000-aa8409ceb2359d903f38 | View in MoNA |
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| Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS | Not Available |
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| GC-MS | GC-MS Spectrum - GC-EI-TOF | splash10-00ks-7930000000-4e9cfd715fa884d30e7f | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-EI-TOF | splash10-00ks-7930000000-4e9cfd715fa884d30e7f | View in MoNA |
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| GC-MS | GC-MS Spectrum - GC-EI-TOF | splash10-000i-9500000000-709ebcb57f006703c269 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated) | splash10-0006-9000000000-bed7ac074a60210971e3 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated) | splash10-014i-9200000000-2fa08d2b4df406cf63ea | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated) | splash10-014j-9000000000-045be8e8dca3f93d45ba | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative | splash10-004i-0900000000-237fa8ad6bfb929be31e | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative | splash10-004r-9700000000-8d38d24fce78b1ff13e6 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative | splash10-0a59-9000000000-759cfc0817917283d1a5 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative | splash10-0a4l-9000000000-2a068ada71fbe0a5a988 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative | splash10-0a4l-9000000000-58e61e31f3c96d84d799 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT , negative | splash10-004i-0900000000-556a0d1cf568609752d1 | View in MoNA |
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| LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT , negative | splash10-004r-5900000000-20f6923c8ed45e9fa088 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-08gr-7900000000-31021fda89eb6535185f | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-0btm-9200000000-3bf5df4b557ef87267f4 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-0a4i-9000000000-37839bf70647d24b94a4 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-004i-4900000000-796467909d1d4c087a07 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-01ri-9400000000-0abcd021721f32c10e42 | View in MoNA |
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| Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-015l-9000000000-5c33a47dfeb09a182983 | View in MoNA |
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| 1D NMR | 1H NMR Spectrum | Not Available |
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| 1D NMR | 1H NMR Spectrum | Not Available |
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| 1D NMR | 13C NMR Spectrum | Not Available |
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| 2D NMR | [1H,13C] 2D NMR Spectrum | Not Available |
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| General References | - Martin PM, Gopal E, Ananth S, Zhuang L, Itagaki S, Prasad BM, Smith SB, Prasad PD, Ganapathy V: Identity of SMCT1 (SLC5A8) as a neuron-specific Na+-coupled transporter for active uptake of L-lactate and ketone bodies in the brain. J Neurochem. 2006 Jul;98(1):279-88. [PubMed:16805814 ]
- Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
- Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. [PubMed:15930465 ]
- Chow LS, Albright RC, Bigelow ML, Toffolo G, Cobelli C, Nair KS: Mechanism of insulin's anabolic effect on muscle: measurements of muscle protein synthesis and breakdown using aminoacyl-tRNA and other surrogate measures. Am J Physiol Endocrinol Metab. 2006 Oct;291(4):E729-36. Epub 2006 May 16. [PubMed:16705065 ]
- Wang Y, Holmes E, Nicholson JK, Cloarec O, Chollet J, Tanner M, Singer BH, Utzinger J: Metabonomic investigations in mice infected with Schistosoma mansoni: an approach for biomarker identification. Proc Natl Acad Sci U S A. 2004 Aug 24;101(34):12676-81. Epub 2004 Aug 16. [PubMed:15314235 ]
- Mitch WE, Walser M, Sapir DG: Nitrogen sparing induced by leucine compared with that induced by its keto analogue, alpha-ketoisocaproate, in fasting obese man. J Clin Invest. 1981 Feb;67(2):553-62. [PubMed:7462428 ]
- Sgaravatti AM, Rosa RB, Schuck PF, Ribeiro CA, Wannmacher CM, Wyse AT, Dutra-Filho CS, Wajner M: Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta. 2003 Nov 20;1639(3):232-8. [PubMed:14636955 ]
- Schadewaldt P, Hammen HW, Ott AC, Wendel U: Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. J Inherit Metab Dis. 1999 Aug;22(6):706-22. [PubMed:10472531 ]
- Hachey DL, Patterson BW, Reeds PJ, Elsas LJ: Isotopic determination of organic keto acid pentafluorobenzyl esters in biological fluids by negative chemical ionization gas chromatography/mass spectrometry. Anal Chem. 1991 May 1;63(9):919-23. [PubMed:1858984 ]
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