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151 related items for PubMed ID: 8537366

  • 1. Roles of amino acid residues surrounding phosphorylation site 1 of branched-chain alpha-ketoacid dehydrogenase (BCKDH) in catalysis and phosphorylation site recognition by BCKDH kinase.
    Hawes JW, Schnepf RJ, Jenkins AE, Shimomura Y, Popov KM, Harris RA.
    J Biol Chem; 1995 Dec 29; 270(52):31071-6. PubMed ID: 8537366
    [Abstract] [Full Text] [Related]

  • 2. Studies on the regulation of the mitochondrial alpha-ketoacid dehydrogenase complexes and their kinases.
    Harris RA, Hawes JW, Popov KM, Zhao Y, Shimomura Y, Sato J, Jaskiewicz J, Hurley TD.
    Adv Enzyme Regul; 1997 Dec 29; 37():271-93. PubMed ID: 9381974
    [Abstract] [Full Text] [Related]

  • 3. Site-directed mutagenesis of phosphorylation sites of the branched chain alpha-ketoacid dehydrogenase complex.
    Zhao Y, Hawes J, Popov KM, Jaskiewicz J, Shimomura Y, Crabb DW, Harris RA.
    J Biol Chem; 1994 Jul 15; 269(28):18583-7. PubMed ID: 8034607
    [Abstract] [Full Text] [Related]

  • 4. Impaired assembly of E1 decarboxylase of the branched-chain alpha-ketoacid dehydrogenase complex in type IA maple syrup urine disease.
    Wynn RM, Davie JR, Chuang JL, Cote CD, Chuang DT.
    J Biol Chem; 1998 May 22; 273(21):13110-8. PubMed ID: 9582350
    [Abstract] [Full Text] [Related]

  • 5. Regulation of the branched-chain alpha-ketoacid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease.
    Harris RA, Zhang B, Goodwin GW, Kuntz MJ, Shimomura Y, Rougraff P, Dexter P, Zhao Y, Gibson R, Crabb DW.
    Adv Enzyme Regul; 1990 May 22; 30():245-63. PubMed ID: 2403034
    [Abstract] [Full Text] [Related]

  • 6. Regulation of branched-chain alpha-ketoacid dehydrogenase complex by covalent modification.
    Harris RA, Paxton R, Powell SM, Goodwin GW, Kuntz MJ, Han AC.
    Adv Enzyme Regul; 1986 May 22; 25():219-37. PubMed ID: 3028049
    [Abstract] [Full Text] [Related]

  • 7. Purification, characterization, regulation and molecular cloning of mitochondrial protein kinases.
    Harris RA, Popov KM, Shimomura Y, Zhao Y, Jaskiewicz J, Nanaumi N, Suzuki M.
    Adv Enzyme Regul; 1992 May 22; 32():267-84. PubMed ID: 1496922
    [Abstract] [Full Text] [Related]

  • 8. A new family of protein kinases--the mitochondrial protein kinases.
    Harris RA, Popov KM, Zhao Y, Kedishvili NY, Shimomura Y, Crabb DW.
    Adv Enzyme Regul; 1995 May 22; 35():147-62. PubMed ID: 7572341
    [Abstract] [Full Text] [Related]

  • 9. Site-directed mutagenesis and functional analysis of the active-site residues of the E2 component of bovine branched-chain alpha-keto acid dehydrogenase complex.
    Meng M, Chuang DT.
    Biochemistry; 1994 Nov 01; 33(43):12879-85. PubMed ID: 7947694
    [Abstract] [Full Text] [Related]

  • 10. Nucleotide and deduced amino acid sequence of the E1 alpha subunit of human liver branched-chain alpha-ketoacid dehydrogenase.
    Zhang B, Crabb DW, Harris RA.
    Gene; 1988 Sep 15; 69(1):159-64. PubMed ID: 3224821
    [Abstract] [Full Text] [Related]

  • 11. Maple syrup urine disease. Complete primary structure of the E1 beta subunit of human branched chain alpha-ketoacid dehydrogenase complex deduced from the nucleotide sequence and a gene analysis of patients with this disease.
    Nobukuni Y, Mitsubuchi H, Endo F, Akaboshi I, Asaka J, Matsuda I.
    J Clin Invest; 1990 Jul 15; 86(1):242-7. PubMed ID: 2365818
    [Abstract] [Full Text] [Related]

  • 12. Ca2+-dependent inhibition of branched-chain α-ketoacid dehydrogenase kinase by thiamine pyrophosphate.
    Noguchi S, Kondo Y, Ito R, Katayama T, Kazama S, Kadota Y, Kitaura Y, Harris RA, Shimomura Y.
    Biochem Biophys Res Commun; 2018 Oct 12; 504(4):916-920. PubMed ID: 30224059
    [Abstract] [Full Text] [Related]

  • 13. Maple syrup urine disease. Complete defect of the E1 beta subunit of the branched chain alpha-ketoacid dehydrogenase complex due to a deletion of an 11-bp repeat sequence which encodes a mitochondrial targeting leader peptide in a family with the disease.
    Nobukuni Y, Mitsubuchi H, Akaboshi I, Indo Y, Endo F, Yoshioka A, Matsuda I.
    J Clin Invest; 1991 May 12; 87(5):1862-6. PubMed ID: 2022752
    [Abstract] [Full Text] [Related]

  • 14. Sequence of the E1 alpha subunit of branched-chain alpha-ketoacid dehydrogenase in two patients with thiamine-responsive maple syrup urine disease.
    Zhang B, Wappner RS, Brandt IK, Harris RA, Crabb DW.
    Am J Hum Genet; 1990 Apr 12; 46(4):843-6. PubMed ID: 2316528
    [Abstract] [Full Text] [Related]

  • 15. Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.
    Wynn RM, Ho R, Chuang JL, Chuang DT.
    J Biol Chem; 2001 Feb 09; 276(6):4168-74. PubMed ID: 11069910
    [Abstract] [Full Text] [Related]

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  • 17. Activation of branched-chain alpha-ketoacid dehydrogenase in isolated hepatocytes by branched-chain alpha-ketoacids.
    Han AC, Goodwin GW, Paxton R, Harris RA.
    Arch Biochem Biophys; 1987 Oct 09; 258(1):85-94. PubMed ID: 3662542
    [Abstract] [Full Text] [Related]

  • 18. Nucleotide sequence of the 5' end including the initiation codon of cDNA for the E1 alpha subunit of the human branched chain alpha-ketoacid dehydrogenase complex.
    McKean MC, Winkeler KA, Danner DJ.
    Biochim Biophys Acta; 1992 Nov 15; 1171(1):109-12. PubMed ID: 1420356
    [Abstract] [Full Text] [Related]

  • 19. Isolation and characterization of a complementary DNA clone coding for the E1 beta subunit of the bovine branched-chain alpha-ketoacid dehydrogenase complex: complete amino acid sequence of the precursor protein and its proteolytic processing.
    Nobukuni Y, Mitsubuchi H, Endo F, Asaka J, Oyama R, Titani K, Matsuda I.
    Biochemistry; 1990 Feb 06; 29(5):1154-60. PubMed ID: 2322554
    [Abstract] [Full Text] [Related]

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