BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

421 related articles for article (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
    [TBL] [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; 265(5):2425-7. PubMed ID: 2303405
    [TBL] [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; 263(18):9007-14. PubMed ID: 3379058
    [TBL] [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; 8(1):39-47. PubMed ID: 1943689
    [TBL] [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; 86(1):242-7. PubMed ID: 2365818
    [TBL] [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; 273(21):13110-8. PubMed ID: 9582350
    [TBL] [Abstract][Full Text] [Related]  

  • 7. 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; 46(4):843-6. PubMed ID: 2316528
    [TBL] [Abstract][Full Text] [Related]  

  • 8. Nutritional and hormonal regulation of the activity state of hepatic branched-chain alpha-keto acid dehydrogenase complex.
    Harris RA; Goodwin GW; Paxton R; Dexter P; Powell SM; Zhang B; Han A; Shimomura Y; Gibson R
    Ann N Y Acad Sci; 1989; 573():306-13. PubMed ID: 2634349
    [TBL] [Abstract][Full Text] [Related]  

  • 9. cDNA cloning of the E1 alpha subunit of the branched-chain alpha-keto acid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease.
    Zhang B; Kuntz MJ; Goodwin GW; Edenberg HJ; Crabb DW; Harris RA
    Ann N Y Acad Sci; 1989; 573():130-6. PubMed ID: 2634344
    [TBL] [Abstract][Full Text] [Related]  

  • 10. 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; 37():271-93. PubMed ID: 9381974
    [TBL] [Abstract][Full Text] [Related]  

  • 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; 87(5):1862-6. PubMed ID: 2022752
    [TBL] [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; 35():147-62. PubMed ID: 7572341
    [TBL] [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; 267(23):16601-6. PubMed ID: 1644840
    [TBL] [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; 25():219-37. PubMed ID: 3028049
    [TBL] [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; 83(4):1425-9. PubMed ID: 2703538
    [TBL] [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; 172(2):646-51. PubMed ID: 2241958
    [TBL] [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; 32():267-84. PubMed ID: 1496922
    [TBL] [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; 243(2):542-55. PubMed ID: 4083900
    [TBL] [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; 264(6):3448-53. PubMed ID: 2914958
    [TBL] [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; 8(3):277-91. PubMed ID: 10745006
    [TBL] [Abstract][Full Text] [Related]  

    [Next]    [New Search]
    of 22.