Record Information |
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Version | 2.0 |
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Created at | 2005-11-16 15:48:42 UTC |
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Updated at | 2021-10-07 20:39:42 UTC |
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NP-MRD ID | NP0000697 |
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Secondary Accession Numbers | None |
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Natural Product Identification |
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Common Name | L-Alloisoleucine |
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Description | L-alloisoleucine is a branched chain amino acid and is a stereo-isomer of L-isoleucine. It is a common constituent of human plasma (albeit at low levels). L-alloisoleucine is produced as a byproduct of isoleucine transamination. L-Isoleucine is one of the four common amino acids (the 3 others being threonine, hydroxyproline, and hydroxylysine) that have two asymmetric carbon atoms which produce four structural possibilities for the same chemical composition. L-alloisoleucine differs from L-isoleucine by having having different stereochemistry around its beta carbon. Plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, can be used for the diagnosis of maple syrup urine disease (MSUD), a genetic disorder. Indeed, plasma L-alloisoleucine levels above 5 umol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD (PMID: 10508118 ). In MSUD the degradation of the essential branched-chain L-amino acids leucine, valine, and isoleucine and their derived 2-oxoacids is impaired because of an inherited deficiency in branched-chain 2-oxoacid dehydrogenase complex (EC 1.2.4.4) Activity. The accumulation of branched-chain compounds in blood and other body fluids can exert neurotoxic effects. |
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Structure | CC[C@@H](C)[C@H](N)C(O)=O InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1 |
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Synonyms | Value | Source |
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(2S,3R)-2-Amino-3-methylpentanoic acid | ChEBI | Alle | ChEBI | Allo-L-isoleucine | ChEBI | L(+)-Alloisoleucine | ChEBI | Threo-3-methyl-L-norvaline | ChEBI | Threo-L-isoleucine | ChEBI | (2S,3R)-2-Amino-3-methylpentanoate | Generator | Alloisoleucine | HMDB | Isoleucine | HMDB | Isoleucine, L isomer | HMDB | Isoleucine, L-isomer | HMDB | L-Isoleucine | HMDB | L-Isomer isoleucine | HMDB | (3R)-LS-Isoleucine | HMDB | 2-Amino-3-methyl-[S-(r*,s*)]-pentanoate | HMDB | 2-Amino-3-methyl-[S-(r*,s*)]-pentanoic acid | HMDB | Allo-isoleucine | HMDB | L-Allo-isoleucine | HMDB | [S-(R*,s*)]-2-amino-3-methylpentanoate | HMDB | [S-(R*,s*)]-2-amino-3-methylpentanoic acid | HMDB |
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Chemical Formula | C6H13NO2 |
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Average Mass | 131.1729 Da |
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Monoisotopic Mass | 131.09463 Da |
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IUPAC Name | (2S,3R)-2-amino-3-methylpentanoic acid |
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Traditional Name | allo-isoleucine |
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CAS Registry Number | 1509-34-8 |
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SMILES | CC[C@@H](C)[C@H](N)C(O)=O |
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InChI Identifier | InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1 |
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InChI Key | AGPKZVBTJJNPAG-UHNVWZDZSA-N |
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Experimental Spectra |
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| Spectrum Type | Description | Depositor Email | Depositor Organization | Depositor | Deposition Date | View |
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1D NMR | 1H NMR Spectrum (1D, 600 MHz, H2O, experimental) | Wishart Lab | Wishart Lab | David Wishart | 2021-06-20 | View Spectrum | 2D NMR | [1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental) | Wishart Lab | Wishart Lab | David Wishart | 2021-06-20 | View Spectrum |
| Predicted Spectra |
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| Not Available | Chemical Shift Submissions |
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| Not Available | Species |
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Species of Origin | |
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Chemical Taxonomy |
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Description | Belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Carboxylic acids and derivatives |
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Sub Class | Amino acids, peptides, and analogues |
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Direct Parent | Isoleucine and derivatives |
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Alternative Parents | |
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Substituents | - Isoleucine or derivatives
- Alpha-amino acid
- L-alpha-amino acid
- Branched fatty acid
- Methyl-branched fatty acid
- Fatty acid
- Fatty acyl
- Amino acid
- Monocarboxylic acid or derivatives
- Carboxylic acid
- Organic oxide
- Organopnictogen compound
- Primary amine
- Organooxygen compound
- Organonitrogen compound
- Primary aliphatic amine
- Carbonyl group
- Organic oxygen compound
- Amine
- Organic nitrogen compound
- Hydrocarbon derivative
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Physical Properties |
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State | Solid |
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Experimental Properties | Property | Value | Reference |
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Melting Point | 60 - 64 °C | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | 34.4 mg/mL at 25 °C | Not Available | LogP | -1.70 | Hansch CH, Leo A and Hoekman DH. "Exploring QSAR: Hydrophobic, Electronic, and Steric Constraints. Volume 1" ACS Publications (1995). |
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Predicted Properties | |
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General References | - Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
- Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40. [PubMed:10508118 ]
- Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Formation of L-alloisoleucine in vivo: an L-[13C]isoleucine study in man. Pediatr Res. 2000 Feb;47(2):271-7. doi: 10.1203/00006450-200002000-00020. [PubMed:10674358 ]
- Bodner-Leidecker A, Wendel U, Saudubray JM, Schadewaldt P: Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation. J Inherit Metab Dis. 2000 Dec;23(8):805-18. doi: 10.1023/a:1026708618507. [PubMed:11196106 ]
- Mamer OA, Reimer ML: On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans. J Biol Chem. 1992 Nov 5;267(31):22141-7. [PubMed:1429566 ]
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