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Journal Abstract Search

147 related items for PubMed ID: 3071697

  • 1. Enzyme assays with mutant cell lines of maple syrup urine disease.
    Chuang DT, Cox RP.
    Methods Enzymol; 1988; 166():135-46. PubMed ID: 3071697
    [No Abstract] [Full Text] [Related]

  • 2. Diagnosis and mutational analysis of maple syrup urine disease using cell cultures.
    Chuang JL, Chuang DT.
    Methods Enzymol; 2000; 324():413-23. PubMed ID: 10989449
    [No Abstract] [Full Text] [Related]

  • 3. [Maple syrup urine disease: molecular pathology of the branched chain alpha-keto acid dehydrogenase complex].
    Nobukuni Y, Mitsubuchi H, Indo Y, Endo F, Matsuda I.
    Seikagaku; 1992 Feb; 64(2):67-82. PubMed ID: 1593184
    [No Abstract] [Full Text] [Related]

  • 4. Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects.
    Chuang DT, Niu WL, Cox RP.
    Biochem J; 1981 Oct 15; 200(1):59-67. PubMed ID: 6895847
    [Abstract] [Full Text] [Related]

  • 5. Maple syrup urine disease 1954 to 1993.
    Peinemann F, Danner DJ.
    J Inherit Metab Dis; 1994 Oct 15; 17(1):3-15. PubMed ID: 8051937
    [No Abstract] [Full Text] [Related]

  • 6. Complementation analysis in lymphoid cells from five patients with different forms of maple syrup urine disease.
    Jinno Y, Akaboshi I, Matsuda I.
    Hum Genet; 1984 Oct 15; 68(1):54-6. PubMed ID: 6500555
    [Abstract] [Full Text] [Related]

  • 7. Analysis of maple syrup urine disease in cell culture: use of substrates.
    Schadewaldt P, Beck K, Wendel U.
    Clin Chim Acta; 1989 Sep 15; 184(1):47-56. PubMed ID: 2598467
    [Abstract] [Full Text] [Related]

  • 8. Study on established lymphoid cells in maple syrup urine disease. Correlation with clinical heterogeneity.
    Jinno Y, Akaboshi I, Katsuki T, Matsuda I.
    Hum Genet; 1984 Sep 15; 65(4):358-61. PubMed ID: 6693123
    [Abstract] [Full Text] [Related]

  • 9. Gene preference in maple syrup urine disease.
    Nellis MM, Danner DJ.
    Am J Hum Genet; 2001 Jan 15; 68(1):232-7. PubMed ID: 11112664
    [Abstract] [Full Text] [Related]

  • 10. [Maple syrup urine disease].
    Matsuda I.
    Tanpakushitsu Kakusan Koso; 1988 Apr 15; 33(5):585-8. PubMed ID: 3270867
    [No Abstract] [Full Text] [Related]

  • 11. Genetic defects in E3 component of alpha-keto acid dehydrogenase complexes.
    Patel MS, Hong YS, Kerr DS.
    Methods Enzymol; 2000 Apr 15; 324():453-64. PubMed ID: 10989452
    [No Abstract] [Full Text] [Related]

  • 12. Activation of branched-chain alpha-ketoacid dehydrogenase complex by alpha-chloroisocaproate in normal and enzyme-deficient fibroblasts.
    Toshima K, Kuroda Y, Yokota I, Naito E, Ito M, Watanabe T, Takeda E, Miyao M.
    Clin Chim Acta; 1985 Apr 15; 147(2):103-8. PubMed ID: 3987057
    [Abstract] [Full Text] [Related]

  • 13. Maple syrup urine disease in a Bedouin tribe: pre- and postnatal diagnosis.
    Potashnik R, Carmi R, Sofer S, Bashan N, Abeliovich D.
    Isr J Med Sci; 1987 Aug 15; 23(8):886-9. PubMed ID: 3679791
    [Abstract] [Full Text] [Related]

  • 14. 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]

  • 15. 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]

  • 16. 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]

  • 17. The role of thiamin in maple syrup urine disease.
    Elsas LJ, Danner DJ.
    Ann N Y Acad Sci; 1982 Jun 25; 378():404-21. PubMed ID: 7044230
    [No Abstract] [Full Text] [Related]

  • 18. 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 25; 21(9-10):895-905. PubMed ID: 6661177
    [Abstract] [Full Text] [Related]

  • 19. Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.
    Chuang DT, Ku LS, Cox RP.
    Proc Natl Acad Sci U S A; 1982 May 25; 79(10):3300-4. PubMed ID: 6954481
    [Abstract] [Full Text] [Related]

  • 20. A structural abnormality of E1 component of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease.
    Indo Y, Kitano A, Akaboshi I, Endo F, Matsuda I.
    J Inherit Metab Dis; 1987 May 25; 10(3):281-3. PubMed ID: 3123794
    [No Abstract] [Full Text] [Related]

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