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418 related items for PubMed ID: 2403034

  • 1. 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; 30():245-63. PubMed ID: 2403034
    [Abstract] [Full Text] [Related]

  • 2. Premature translation termination of the pre-E1 alpha subunit of the branched chain alpha-ketoacid dehydrogenase as a cause of maple syrup urine disease in Polled Hereford calves.
    Zhang B, Healy PJ, Zhao Y, Crabb DW, Harris RA.
    J Biol Chem; 1990 Feb 15; 265(5):2425-7. PubMed ID: 2303405
    [Abstract] [Full Text] [Related]

  • 3. Isolation and sequencing of a cDNA encoding the decarboxylase (E1)alpha precursor of bovine branched-chain alpha-keto acid dehydrogenase complex. Expression of E1 alpha mRNA and subunit in maple-syrup-urine-disease and 3T3-L1 cells.
    Hu CW, Lau KS, Griffin TA, Chuang JL, Fisher CW, Cox RP, Chuang DT.
    J Biol Chem; 1988 Jun 25; 263(18):9007-14. PubMed ID: 3379058
    [Abstract] [Full Text] [Related]

  • 4. 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 25; 8(1):39-47. PubMed ID: 1943689
    [Abstract] [Full Text] [Related]

  • 5. 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 25; 86(1):242-7. PubMed ID: 2365818
    [Abstract] [Full Text] [Related]

  • 6. 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
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  • 11. 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 22; 87(5):1862-6. PubMed ID: 2022752
    [Abstract] [Full Text] [Related]

  • 12. 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]

  • 13. Expression and assembly of a functional E1 component (alpha 2 beta 2) of mammalian branched-chain alpha-ketoacid dehydrogenase complex in Escherichia coli.
    Davie JR, Wynn RM, Cox RP, Chuang DT.
    J Biol Chem; 1992 Aug 15; 267(23):16601-6. PubMed ID: 1644840
    [Abstract] [Full Text] [Related]

  • 14. 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 Aug 15; 25():219-37. PubMed ID: 3028049
    [Abstract] [Full Text] [Related]

  • 15. Evidence for both a regulatory mutation and a structural mutation in a family with maple syrup urine disease.
    Zhang B, Edenberg HJ, Crabb DW, Harris RA.
    J Clin Invest; 1989 Apr 15; 83(4):1425-9. PubMed ID: 2703538
    [Abstract] [Full Text] [Related]

  • 16. A T-to-A substitution in the E1 alpha subunit gene of the branched-chain alpha-ketoacid dehydrogenase complex in two cell lines derived from Menonite maple syrup urine disease patients.
    Matsuda I, Nobukuni Y, Mitsubuchi H, Indo Y, Endo F, Asaka J, Harada A.
    Biochem Biophys Res Commun; 1990 Oct 30; 172(2):646-51. PubMed ID: 2241958
    [Abstract] [Full Text] [Related]

  • 17. 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 Oct 30; 32():267-84. PubMed ID: 1496922
    [Abstract] [Full Text] [Related]

  • 18. Physiological covalent regulation of rat liver branched-chain alpha-ketoacid dehydrogenase.
    Harris RA, Powell SM, Paxton R, Gillim SE, Nagae H.
    Arch Biochem Biophys; 1985 Dec 30; 243(2):542-55. PubMed ID: 4083900
    [Abstract] [Full Text] [Related]

  • 19. Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex.
    Fisher CW, Chuang JL, Griffin TA, Lau KS, Cox RP, Chuang DT.
    J Biol Chem; 1989 Feb 25; 264(6):3448-53. PubMed ID: 2914958
    [Abstract] [Full Text] [Related]

  • 20. 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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