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135 related items for PubMed ID: 5689906

  • 1. Branched chain alpha-keto acid metabolism. I. Isolation, purification, and partial characterization of bovine liver alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid dehydrogenase.
    Connelly JL, Danner DJ, Bowden JA.
    J Biol Chem; 1968 Mar 25; 243(6):1198-203. PubMed ID: 5689906
    [No Abstract] [Full Text] [Related]

  • 2. Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
    Singh S, Willers I, Goedde HW.
    Clin Genet; 1977 Apr 25; 11(4):277-84. PubMed ID: 192504
    [Abstract] [Full Text] [Related]

  • 3. Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.
    Lancaster G, Mamer OA, Scriver CR.
    Metabolism; 1974 Mar 25; 23(3):257-65. PubMed ID: 4813956
    [No Abstract] [Full Text] [Related]

  • 4. Mammalian alpha-keto acid dehydrogenase complexes. IV. Substrate specificities and kinetic properties of the pig heart pyruvate and 2-oxyoglutarate dehydrogenase complexes.
    Kanzaki T, Hayakawa T, Hamada M, Fukuyoshi Y, Koike M.
    J Biol Chem; 1969 Mar 10; 244(5):1183-7. PubMed ID: 5812959
    [No Abstract] [Full Text] [Related]

  • 5. Assays for E1 and E2 components of the branched-chain keto acid dehydrogenase complex.
    Chuang DT.
    Methods Enzymol; 1988 Mar 10; 166():146-54. PubMed ID: 3071698
    [No Abstract] [Full Text] [Related]

  • 6. Further observations on the biochemical lesion in maple syrup urine disease.
    Dreyfus PM, Prensky AL.
    Nature; 1967 Apr 15; 214(5085):276. PubMed ID: 6034241
    [No Abstract] [Full Text] [Related]

  • 7. Immunosuppressive effects of organic acids accumulating in patients with maple syrup urine disease.
    Wajner M, Schlottfeldt JL, Ckless K, Wannmacher CM.
    J Inherit Metab Dis; 1995 Apr 15; 18(2):165-8. PubMed ID: 7564237
    [No Abstract] [Full Text] [Related]

  • 8. Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.
    Wajner A, Bürger C, Dutra-Filho CS, Wajner M, de Souza Wyse AT, Wannmacher CM.
    Metab Brain Dis; 2007 Mar 15; 22(1):77-88. PubMed ID: 17295076
    [Abstract] [Full Text] [Related]

  • 9. Oxidation of branched chain -ketoacids in Streptococcus faecalis and it's dependence on lipoic acid.
    Rüdiger HW, Langenbeck U, Goedde HW.
    Hoppe Seylers Z Physiol Chem; 1972 Jun 15; 353(6):875-82. PubMed ID: 4626441
    [No Abstract] [Full Text] [Related]

  • 10. Blood and tissue branched-chain amino and alpha-keto acid concentrations: effect of diet, starvation, and disease.
    Hutson SM, Harper AE.
    Am J Clin Nutr; 1981 Feb 15; 34(2):173-83. PubMed ID: 7211722
    [Abstract] [Full Text] [Related]

  • 11. Transaminations between amino acids and keto acids elevated in phenylketonuria and maple syrup urine disease.
    Lees GJ, Weiner N.
    J Neurochem; 1973 Feb 15; 20(2):389-403. PubMed ID: 4698286
    [No Abstract] [Full Text] [Related]

  • 12. Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease.
    Rüdiger HW, Langenbeck U, Schulze-Schencking M, Goedde HW.
    Humangenetik; 1972 Feb 15; 14(4):257-63. PubMed ID: 5051750
    [No Abstract] [Full Text] [Related]

  • 13. Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
    Funchal C, Rosa AM, Wajner M, Wofchuk S, Pureur RP.
    Neurochem Res; 2004 Apr 15; 29(4):747-53. PubMed ID: 15098937
    [Abstract] [Full Text] [Related]

  • 14. alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.
    Bridi R, Braun CA, Zorzi GK, Wannmacher CM, Wajner M, Lissi EG, Dutra-Filho CS.
    Metab Brain Dis; 2005 Jun 15; 20(2):155-67. PubMed ID: 15938133
    [Abstract] [Full Text] [Related]

  • 15. pH regulation of mitochondrial branch chain alpha-keto acid transport and oxidation in rat heart mitochondria.
    Hutson SM.
    J Biol Chem; 1987 Jul 15; 262(20):9629-35. PubMed ID: 3597428
    [Abstract] [Full Text] [Related]

  • 16. Fatty acid-requiring mutant of bacillus subtilis defective in branched chain alpha-keto acid dehydrogenase.
    Willecke K, Pardee AB.
    J Biol Chem; 1971 Sep 10; 246(17):5264-72. PubMed ID: 4999353
    [No Abstract] [Full Text] [Related]

  • 17. Coenzyme A- and nicotinamide adenine dinucleotide-dependent branched chain alpha-keto acid dehydrogenase. I. Purification and properties of the enzyme from Bacillus subtilis.
    Namba Y, Yoshizawa K, Ejima A, Hayashi T, Kaneda T.
    J Biol Chem; 1969 Aug 25; 244(16):4437-47. PubMed ID: 4308861
    [No Abstract] [Full Text] [Related]

  • 18. The enzyme defect in maple syrup urine disease (branched chain ketoaciduria).
    Seegmiller JE, Westall RG.
    J Ment Defic Res; 1967 Dec 25; 11(4):288-94. PubMed ID: 5582930
    [No Abstract] [Full Text] [Related]

  • 19. Branched chain alpha-keto acid oxidative decarboxylation in skeletal muscle mitochondria. Effect of isolation procedure and mitochondrial delta pH.
    Hutson SM.
    J Biol Chem; 1986 Apr 05; 261(10):4420-5. PubMed ID: 3957903
    [Abstract] [Full Text] [Related]

  • 20. The mechanism of alpha-ketoisocaproate oxygenase. Formation of beta-hydroxyisovalerate from alpha-ketoisocaproate.
    Sabourin PJ, Bieber LL.
    J Biol Chem; 1982 Jul 10; 257(13):7468-71. PubMed ID: 7085633
    [Abstract] [Full Text] [Related]

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