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Chemical Compound Review

AGN-PC-004KV7     3-hydroxy-2-methyl-propanoic acid

Synonyms: CHEBI:18064, AC1Q5RQW, CTK1A2390, AR-1H6690, AKOS008135403, ...
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Disease relevance of C01188


High impact information on C01188


Biological context of C01188


Anatomical context of C01188

  • The conversion of 1-14C-leucine and 1-14C-valine to 14CO2 and of U-L-14C-valine to its major metabolic product 3-hydroxyisobutyric acid by fibroblasts derived from the patient was less than 5% of control [8].

Associations of C01188 with other chemical compounds


Gene context of C01188


  1. 3-Hydroxyisobutyrate dehydrogenase, an impurity in commercial 3-hydroxybutyrate dehydrogenase. Worrall, E.B., Gassain, S., Cox, D.J., Sugden, M.C., Palmer, T.N. Biochem. J. (1987) [Pubmed]
  2. Cloning and overexpression of the 3-hydroxyisobutyrate dehydrogenase gene from pseudomonas putida E23. Chowdhury, E.K., Akaishi, Y., Nagata, S., Misono, H. Biosci. Biotechnol. Biochem. (2003) [Pubmed]
  3. Purification and characterization of 3-hydroxyisobutyrate dehydrogenase from rabbit liver. Rougraff, P.M., Paxton, R., Kuntz, M.J., Crabb, D.W., Harris, R.A. J. Biol. Chem. (1988) [Pubmed]
  4. Crystal structure of novel NADP-dependent 3-hydroxyisobutyrate dehydrogenase from Thermus thermophilus HB8. Lokanath, N.K., Ohshima, N., Takio, K., Shiromizu, I., Kuroishi, C., Okazaki, N., Kuramitsu, S., Yokoyama, S., Miyano, M., Kunishima, N. J. Mol. Biol. (2005) [Pubmed]
  5. Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects. Avogaro, A., Bier, D.M. J. Lipid Res. (1989) [Pubmed]
  6. Valine metabolism. Gluconeogenesis from 3-hydroxyisobutyrate. Letto, J., Brosnan, M.E., Brosnan, J.T. Biochem. J. (1986) [Pubmed]
  7. Brain dysgenesis and congenital intracerebral calcification associated with 3-hydroxyisobutyric aciduria. Chitayat, D., Meagher-Villemure, K., Mamer, O.A., O'Gorman, A., Hoar, D.I., Silver, K., Scriver, C.R. J. Pediatr. (1992) [Pubmed]
  8. Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase. Yoshida, I., Sweetman, L., Kulovich, S., Nyhan, W.L., Robinson, B.H. Pediatr. Res. (1990) [Pubmed]
  9. 3-Hydroxyisobutyric aciduria: an inborn error of valine metabolism. Ko, F.J., Nyhan, W.L., Wolff, J., Barshop, B., Sweetman, L. Pediatr. Res. (1991) [Pubmed]
  10. Coordinated expression of valine catabolic enzymes during adipogenesis: analysis of activity, mRNA, protein levels, and metabolic consequences. Kedishvili, N.Y., Popov, K.M., Jaskiewicz, J.A., Harris, R.A. Arch. Biochem. Biophys. (1994) [Pubmed]
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