| 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 | 2023-02-21 17:14:56 UTC |
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| HMDB ID | HMDB0000557 |
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| Secondary Accession Numbers | - HMDB0002291
- HMDB00557
- HMDB02291
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| Metabolite 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 Molecular Weight | 131.1729 |
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| Monoisotopic Molecular Weight | 131.094628665 |
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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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| 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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| Ontology |
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| Not Available | Not Available |
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| Physical Properties |
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| State | Solid |
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| Experimental Molecular 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,C ET AL. (1995) |
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| Experimental Chromatographic Properties | Experimental Collision Cross Sections |
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| Predicted Molecular Properties | |
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| Predicted Chromatographic Properties | Predicted Collision Cross SectionsPredicted Retention Times Underivatized| Chromatographic Method | Retention Time | Reference |
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| 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. | 1.25 minutes | 32390414 | | Predicted by Siyang on May 30, 2022 | 9.9376 minutes | 33406817 | | Predicted by Siyang using ReTip algorithm on June 8, 2022 | 6.56 minutes | 32390414 | | AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid | 317.8 seconds | 40023050 | | Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid | 692.6 seconds | 40023050 | | Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid | 326.9 seconds | 40023050 | | Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid | 74.6 seconds | 40023050 | | Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid | 181.5 seconds | 40023050 | | RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid | 59.7 seconds | 40023050 | | Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid | 305.5 seconds | 40023050 | | BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid | 277.3 seconds | 40023050 | | HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate) | 549.9 seconds | 40023050 | | UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid | 663.4 seconds | 40023050 | | BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid | 149.4 seconds | 40023050 | | UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid | 778.9 seconds | 40023050 | | SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid | 180.2 seconds | 40023050 | | RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid | 238.5 seconds | 40023050 | | MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate | 490.4 seconds | 40023050 | | KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA | 393.3 seconds | 40023050 | | Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water | 252.5 seconds | 40023050 |
Predicted Kovats Retention IndicesUnderivatizedDerivatized| Derivative Name / Structure | SMILES | Kovats RI Value | Column Type | Reference |
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| L-Alloisoleucine,1TMS,isomer #1 | CC[C@@H](C)[C@H](N)C(=O)O[Si](C)(C)C | 1180.4 | Semi standard non polar | 33892256 | | L-Alloisoleucine,1TMS,isomer #2 | CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O | 1292.1 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C | 1316.6 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C | 1308.4 | Standard non polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C | 1419.5 | Standard polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C | 1480.8 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C | 1322.9 | Standard non polar | 33892256 | | L-Alloisoleucine,2TMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C | 1511.7 | Standard polar | 33892256 | | L-Alloisoleucine,3TMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C | 1501.3 | Semi standard non polar | 33892256 | | L-Alloisoleucine,3TMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C | 1434.9 | Standard non polar | 33892256 | | L-Alloisoleucine,3TMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C | 1401.2 | Standard polar | 33892256 | | L-Alloisoleucine,1TBDMS,isomer #1 | CC[C@@H](C)[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C | 1396.9 | Semi standard non polar | 33892256 | | L-Alloisoleucine,1TBDMS,isomer #2 | CC[C@@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O | 1519.3 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C | 1749.9 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C | 1724.7 | Standard non polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #1 | CC[C@@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C | 1722.9 | Standard polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 1878.2 | Semi standard non polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 1758.8 | Standard non polar | 33892256 | | L-Alloisoleucine,2TBDMS,isomer #2 | CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 1750.4 | Standard polar | 33892256 | | L-Alloisoleucine,3TBDMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 2161.4 | Semi standard non polar | 33892256 | | L-Alloisoleucine,3TBDMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 2055.1 | Standard non polar | 33892256 | | L-Alloisoleucine,3TBDMS,isomer #1 | CC[C@@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C | 1828.2 | Standard polar | 33892256 |
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| Disease References | | Maple syrup urine disease |
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- 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 ]
- Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ]
| | Short/branched chain acyl-CoA dehydrogenase deficiency |
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- 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 ]
| | Colorectal cancer |
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- 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 ]
| | Eosinophilic esophagitis |
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- Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
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