References
- Dancis J, Hutzler J, Levitz M. Metabolism of the white blood cells in maple syrup urine disease. Biochim Biophys Acta 1960; 43: 342–3
- Langenbeck U, Rüdiger H W, Schulze-Schencking M, Keller W, Brackerts D, Goedde H W. Evaluation of a heterozygote test for maple syrup urine disease in leukocytes and cultured fibroblasts. Humangenetik 1971; 11: 304–15
- Paul H S, Adibi S A. Regulation of branched chain amino acid catabolism. Branched Chain Amino and Keto Acids in Health and Disease, S A Adibi, W Fekl, U Langenbeck, P Schauder. Karger, Basel 1984; 182–219
- Wendel U, Wöhler W, Goedde H W, Langenbeck U, Passarge E, Rüdiger H W. Rapid diagnosis of maple syrup urine disease (branched chain ketoacidosis) by micro-enzymne assay in leukocytes and fibroblasts. Clin Chim Acta 1973; 45: 433–40
- Rändle P J. Mitochondrial 2-oxoacid dehydrogenase complexes of animal tissues. Phil Trans R Soc Lond (B) 1983; 302: 47–57
- Rüdiger H W, Langenbeck U, Goedde H W. A simplified method for the preparation of 14C-labeled branched-chain α-oxo acids. Biochem J 1972; 126: 445–6
- Bøyum A. Isolation of mononuclear cells and granulocytes from human blood. IV. Scand J Clin Lab Invest 1968; 21((Suppl. 97))77–89
- Wagenmakers A JM, Schepens J TG, Veldhuizen J AM, Veerkamp J H. The activity state of the branched-chain 2-oxoacid dehydrogenase complex in rat tissues. Biochem J 1984; 220: 273–81
- Linn T C, Telley J W, Pettit F H, Hucho F, Randall D D, Reed L J. α-keto acid dehydrogenase complex XI. Purification and properties of the component enzymes of the pyruvate dehydrogenase complexes from bovine kidney and heart. Arch Biochem Biophys 1972; 148: 326–42
- Lau K S, Fatania H R, Randle P J. Regulation of the branched chain 2-oxoacid dehydrogenase kinase reaction. FEBS Lett 1982; 144: 57–62
- Lenzen S, Panten U. 2-oxocarboxylic acids and function of pancreatic islets in obese-hyperglycemic mice. Biochem J 1980; 186: 135–44
- Paul H S, Adibi S A. Assessment of effect of starvation glucose, fatty acids and hormones on α-decarboxylation of leucine in skeletal muscle of rat. J Nutr 1976; 106: 1079–88
- Bremer H J, Duran M, Kamerling J P, Przyrembel H, Wadman S K. Disturbances of Amino Acid metabolism: Clinical Chemistry and Diagnosis. Urban & Schwarzenberg, München 1981; 203–7
- Schauder P. Effect of starvation, dietary protein, and pancreatic hormones on branched chain keto acid blood levels in man. Branched Chain Amino and Keto Acids in Health and Disease, S A Adibi, W Fekl, U Langenbeck, P Schauder. Karger, Basel 1984; 228–41
- Frick G P, Tai L R, Blinder L, Goodman H M. L-leucine activates branched-chain keto acid dehydrogenase in rat adipose tissue. J Biol Chem 1981; 256: 2618–20
- Harris R A, Paxton R, DePaoli-Roach A A. Inhibition of branched chain α-ketoacid dehydrogenase kinase activity by α-chloroisocaproate. J Biol Chem 1981; 257: 13915–8
- Paxton R, Harris R A. Isolation of rabbit liver branched chain α-ketoacid dehydrogenase and regulation by phosphorylation. J Biol Chem 1982; 257: 14433–9