BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

144 related articles for article (PubMed ID: 1644840)

  • 1. 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]  

  • 2. Chaperonins groEL and groES promote assembly of heterotetramers (alpha 2 beta 2) of mammalian mitochondrial branched-chain alpha-keto acid decarboxylase in Escherichia coli.
    Wynn RM; Davie JR; Cox RP; Chuang DT
    J Biol Chem; 1992 Jun; 267(18):12400-3. PubMed ID: 1352285
    [TBL] [Abstract][Full Text] [Related]  

  • 3. 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]  

  • 4. 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]  

  • 5. Cloning and expression in Escherichia coli of mature E1 beta subunit of bovine mitochondrial branched-chain alpha-keto acid dehydrogenase complex. Mapping of the E1 beta-binding region on E2.
    Wynn RM; Chuang JL; Davie JR; Fisher CW; Hale MA; Cox RP; Chuang DT
    J Biol Chem; 1992 Jan; 267(3):1881-7. PubMed ID: 1730724
    [TBL] [Abstract][Full Text] [Related]  

  • 6. 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]  

  • 7. 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]  

  • 8. 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]  

  • 9. Molecular basis of maple syrup urine disease: novel mutations at the E1 alpha locus that impair E1(alpha 2 beta 2) assembly or decrease steady-state E1 alpha mRNA levels of branched-chain alpha-keto acid dehydrogenase complex.
    Chuang JL; Fisher CR; Cox RP; Chuang DT
    Am J Hum Genet; 1994 Aug; 55(2):297-304. PubMed ID: 8037208
    [TBL] [Abstract][Full Text] [Related]  

  • 10. 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]  

  • 11. 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]  

  • 12. Maple syrup urine disease in Mennonites. Evidence that the Y393N mutation in E1 alpha impedes assembly of the E1 component of branched-chain alpha-keto acid dehydrogenase complex.
    Fisher CR; Chuang JL; Cox RP; Fisher CW; Star RA; Chuang DT
    J Clin Invest; 1991 Sep; 88(3):1034-7. PubMed ID: 1885764
    [TBL] [Abstract][Full Text] [Related]  

  • 13. Stable correction of maple syrup urine disease in cells from a Mennonite patient by retroviral-mediated gene transfer.
    Koyata H; Cox RP; Chuang DT
    Biochem J; 1993 Nov; 295 ( Pt 3)(Pt 3):635-9. PubMed ID: 8240269
    [TBL] [Abstract][Full Text] [Related]  

  • 14. 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]  

  • 15. Heterogeneity of mutations in maple syrup urine disease (MSUD): screening and identification of affected E1 alpha and E1 beta subunits of the branched-chain alpha-keto-acid dehydrogenase multienzyme complex.
    Nobukuni Y; Mitsubuchi H; Hayashida Y; Ohta K; Indo Y; Ichiba Y; Endo F; Matsuda I
    Biochim Biophys Acta; 1993 Nov; 1225(1):64-70. PubMed ID: 8161368
    [TBL] [Abstract][Full Text] [Related]  

  • 16. Cloning and sequencing of a cluster of genes encoding branched-chain alpha-keto acid dehydrogenase from Streptomyces avermitilis and the production of a functional E1 [alpha beta] component in Escherichia coli.
    Skinner DD; Morgenstern MR; Fedechko RW; Denoya CD
    J Bacteriol; 1995 Jan; 177(1):183-90. PubMed ID: 8002616
    [TBL] [Abstract][Full Text] [Related]  

  • 17. 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; 8(1):49-63. PubMed ID: 1943690
    [TBL] [Abstract][Full Text] [Related]  

  • 18. Molecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patients.
    Chuang JL; Davie JR; Chinsky JM; Wynn RM; Cox RP; Chuang DT
    J Clin Invest; 1995 Mar; 95(3):954-63. PubMed ID: 7883996
    [TBL] [Abstract][Full Text] [Related]  

  • 19. Occurrence of a Tyr393----Asn (Y393N) mutation in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients from a Mennonite population.
    Fisher CR; Fisher CW; Chuang DT; Cox RP
    Am J Hum Genet; 1991 Aug; 49(2):429-34. PubMed ID: 1867199
    [TBL] [Abstract][Full Text] [Related]  

  • 20. 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]  

    [Next]    [New Search]
    of 8.