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127 related items for PubMed ID: 11069910

  • 1. 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]

  • 2. Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
    AEvarsson A, Chuang JL, Wynn RM, Turley S, Chuang DT, Hol WG.
    Structure; 2000 Mar 15; 8(3):277-91. PubMed ID: 10745006
    [Abstract] [Full Text] [Related]

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  • 4. Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.
    Wynn RM, Chuang JL, Sansaricq C, Mandel H, Chuang DT.
    J Biol Chem; 2001 Sep 28; 276(39):36550-6. PubMed ID: 11448970
    [Abstract] [Full Text] [Related]

  • 5. 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]

  • 6. Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease.
    Song JL, Chuang DT.
    J Biol Chem; 2001 Oct 26; 276(43):40241-6. PubMed ID: 11507102
    [Abstract] [Full Text] [Related]

  • 7. Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis.
    Turchany JM, Leung PS, Iwayama T, Jefferson DM, Ishida J, Yamaguchi M, Munoz S, Danner DJ, Dickson ER, Gershwin ME.
    J Autoimmun; 1993 Aug 26; 6(4):459-66. PubMed ID: 8216688
    [Abstract] [Full Text] [Related]

  • 8. 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 Aug 26; 30():245-63. PubMed ID: 2403034
    [Abstract] [Full Text] [Related]

  • 9. Tetrameric assembly and conservation in the ATP-binding domain of rat branched-chain alpha-ketoacid dehydrogenase kinase.
    Wynn RM, Chuang JL, Cote CD, Chuang DT.
    J Biol Chem; 2000 Sep 29; 275(39):30512-9. PubMed ID: 10903321
    [Abstract] [Full Text] [Related]

  • 10. Branched-chain ketoacid dehydrogenase activity and growth of normal and mutant human fibroblasts: the effect of branched-chain amino acid concentration in culture medium.
    Danner DJ, Priest JH.
    Biochem Genet; 1983 Oct 29; 21(9-10):895-905. PubMed ID: 6661177
    [Abstract] [Full Text] [Related]

  • 11. Maple syrup urine disease: it has come a long way.
    Chuang DT.
    J Pediatr; 1998 Mar 29; 132(3 Pt 2):S17-23. PubMed ID: 9546032
    [Abstract] [Full Text] [Related]

  • 12. 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]

  • 13. Roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site.
    Wynn RM, Machius M, Chuang JL, Li J, Tomchick DR, Chuang DT.
    J Biol Chem; 2003 Oct 31; 278(44):43402-10. PubMed ID: 12902323
    [Abstract] [Full Text] [Related]

  • 14. Folding kinetics of the lipoic acid-bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase complex.
    Naik MT, Chang YC, Huang TH.
    FEBS Lett; 2002 Oct 23; 530(1-3):133-8. PubMed ID: 12387880
    [Abstract] [Full Text] [Related]

  • 15. Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells.
    Nellis MM, Doering CB, Kasinski A, Danner DJ.
    Am J Physiol Endocrinol Metab; 2002 Oct 23; 283(4):E853-60. PubMed ID: 12217904
    [Abstract] [Full Text] [Related]

  • 16. Gene preference in maple syrup urine disease.
    Nellis MM, Danner DJ.
    Am J Hum Genet; 2001 Jan 23; 68(1):232-7. PubMed ID: 11112664
    [Abstract] [Full Text] [Related]

  • 17. 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 Jan 23; 25():219-37. PubMed ID: 3028049
    [Abstract] [Full Text] [Related]

  • 18. Molecular defects in the E1 alpha subunit of the branched-chain alpha-ketoacid dehydrogenase complex that cause maple syrup urine disease.
    Zhang B, Zhao Y, Harris RA, Crabb DW.
    Mol Biol Med; 1991 Feb 23; 8(1):39-47. PubMed ID: 1943689
    [Abstract] [Full Text] [Related]

  • 19. Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex.
    Chuang DT, Fisher CW, Lau KS, Griffin TA, Wynn RM, Cox RP.
    Mol Biol Med; 1991 Feb 23; 8(1):49-63. PubMed ID: 1943690
    [Abstract] [Full Text] [Related]

  • 20. E2 transacylase-deficient (type II) maple syrup urine disease. Aberrant splicing of E2 mRNA caused by internal intronic deletions and association with thiamine-responsive phenotype.
    Chuang JL, Cox RP, Chuang DT.
    J Clin Invest; 1997 Aug 01; 100(3):736-44. PubMed ID: 9239422
    [Abstract] [Full Text] [Related]

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