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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]
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]