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Chemical Compound Review

Isoleucine     2-amino-3-methyl-pentanoic acid

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Disease relevance of Isoleucine


High impact information on Isoleucine

  • As judged from the fictive initial concentration, L-alloisoleucine is distributed evenly in the total body water space [6].
  • When human skin fibroblast cultures were challenged with L-isoleucine, KMV accumulated at a gradually decreased rate, whereas L-alloisoleucine accumulated at a gradually accelerated rate [7].
  • The relatively small difference between normal and MSUD fibroblasts in vitro as opposed to the striking differences between healthy subjects and MSUD patients in vivo are discussed with respect to the significance of physiological mechanisms participating in the formation and degradation of L-alloisoleucine in man [7].
  • Branched-chain amino acid interactions in skeletal muscle: isoleucine and L-alloisoleucine [8].
  • Parenteral administration of a mixture of branched-chain amino acid (BCAA) solutions is known to alter plasma levels of the BCAA (ILE, LEU, VAL), their corresponding alpha-ketoacids (KMV, KIC, KIV) and L-alloisoleucine (ALLO), a stereoisomer of ILE [8].

Chemical compound and disease context of Isoleucine


Biological context of Isoleucine


Anatomical context of Isoleucine


Associations of Isoleucine with other chemical compounds


Analytical, diagnostic and therapeutic context of Isoleucine

  • Furthermore, the spontaneous resolution of enantiomers upon crystallization of the other racemate, DL-allo-isoleucine, is rationalized on the basis of the aforementioned analysis of interatomic distances in the 1:1 DL-isoleucine:DL-allo-isoleucine complex [13].


  1. 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. Mamer, O.A., Reimer, M.L. J. Biol. Chem. (1992) [Pubmed]
  2. Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Schadewaldt, P., Bodner-Leidecker, A., Hammen, H.W., Wendel, U. Clin. Chem. (1999) [Pubmed]
  3. Atypical phenotype in a boy with a maple syrup urine disease. Ben-Omran, T.I., Blaser, S., Phillips, H., Callahan, J., Feigenbaum, A. J. Inherit. Metab. Dis. (2006) [Pubmed]
  4. Leigh disease with deficiency of lipoamide dehydrogenase: treatment failure with dichloroacetate. Craigen, W.J. Pediatric neurology. (1996) [Pubmed]
  5. Isoleucine, an essential amino acid, prevents liver metastases of colon cancer by antiangiogenesis. Murata, K., Moriyama, M. Cancer Res. (2007) [Pubmed]
  6. Oral L-alloisoleucine loading studies in healthy subjects and in patients with maple syrup urine disease. Schadewaldt, P., Dalle-Feste, C., Langenbeck, U., Wendel, U. Pediatr. Res. (1991) [Pubmed]
  7. On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease. Schadewaldt, P., Hammen, H.W., Dalle-Feste, C., Wendel, U. J. Inherit. Metab. Dis. (1990) [Pubmed]
  8. Branched-chain amino acid interactions in skeletal muscle: isoleucine and L-alloisoleucine. Downey, R.S., Karl, I.E., Bier, D.M. JPEN. Journal of parenteral and enteral nutrition. (1986) [Pubmed]
  9. Alloisoleucine formation in maple syrup urine disease: isotopic evidence for the mechanism. Matthews, D.E., Ben-Galim, E., Haymond, M.W., Bier, D.M. Pediatr. Res. (1980) [Pubmed]
  10. Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease. Wendel, U., Even, G., Langenbeck, U., Schadewaldt, P., Hummel, W. Clin. Chim. Acta (1992) [Pubmed]
  11. Transamination and oxidative decarboxylation of L-isoleucine, L-alloisoleucine and related 2-oxo acids in perfused rat hind limb muscle. Schadewaldt, P., Radeck, W., Hammen, H.W., Staib, W. Biochim. Biophys. Acta (1989) [Pubmed]
  12. Plasma alloisoleucine: analytical method and clearance in ketoacid-supplemented normals. Ponto, K.H., Anderson, P.A., Kies, C.V. Kidney Int. Suppl. (1989) [Pubmed]
  13. Structural relationships in crystals accommodating different stereoisomers of 2-amino-3-methylpentanoic acid. Dalhus, B., Gorbitz, C.H. Acta Crystallogr., B (2000) [Pubmed]
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