Biochemical Studies
Jaskiewicz, J., K.M. Popov, and R.A. Harris, 1995 Alpha-ketoisocaproate dioxygenase, a dual-specificity enzyme, corresponds to alloantigen F, FASEB J. 9:A1318
Nissen, S., M. Van Koevering, and D. Webb, 1990 Analysis of ß-hydroxy-ß-methylbutyrate in plasma by gas chromatography and mass spectrometry, Anal. Biochem. 188:17-19
Hokland, B.M. and J. Bremer, 1988 Formation and excretion of branched-chain acylcarnitines and branched-chain hydroxy acids in the perfused rat kidney, Biochim. Biophys. Acta. 961:30-37
Wagenmakers, A.J.M., H.J.M. Salden and J.H. Veerkamp, 1985 The metabolic fate of branched chain amino acids and 2-oxo acids in rat muscle homogenates and diaphragms, Int. J. Biochem. 17:957-965
Sabourin, P.J. and L.L. Bieber, 1983 Formation of beta-hydroxyisovalerate by an alpha-ketoisocaproate oxygenase in human liver, Metab. 32:160-164
Spydevold, O. and B. Hokland, 1983 Release of leucine and isoleucine metabolites by perfused skeletal muscle and liver of rat, Int. J. Biochem. 15:985-990
Sabourin, P.J. and L.L. Bieber, 1982 The mechanism of alpha-ketoisocaproate oxygenase. Formation of ß-hydroxyisovalerate from alpha-ketoisocaproate, J. Biol. Chem. 257:7468-7471
Sabourin, P.J. and L.L. Bieber, 1982 Purification and characterization of an alpha-ketoisocaproate oxygenase of rat liver, J. Biol. Chem. 257:7460-7467
Sabourin, P.J. and L.L. Bieber, 1981 Subcellular distribution and partial characterization of an alpha-ketoisocaproate oxidase of rat liver: formation of ß-hydroxyisovaleric acid, Arch. Biochem. Biophys. 206:132-144
Sabourin, P.J. and L.L. Bieber, 1981 Formation of ß-hydroxyisovalerate from alpha-ketoisocaproate by a soluble preparation from rat liver, Dev. Biochem. 18:149-154
Packman, S., L.D. Sweetman, M. Yoshino, H. Baker, and M. Cowan, 1981 Biotin-responsive multiple carboxylase deficiency of infantile onset, J. Pediatr. 99:421-423
Sabourin, P.J. and L.L. Bieber, 1979 Branched-chain alpha-keto acid decarboxylases in rat liver, Fed. Proc. 38:283
Adamson, L.F. and D.M. Greenberg, 1957 The significance of certain carboxylic acids as intermediates in the biosynthesis of cholesterol, Biochimica et Biophysica Acta 23:472-479
Rudney, H. and T.G. Farkas, 1955 Biosynthesis of branched chain acids, Fed. Proc. 15:757-759
Rubinus, J.L., 1955 The biosynthesis of radioactive ß-hydroxyisovaleric acid in rat liver, J. Am. Chem. Soc. 77:1295-1297