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186 related items for PubMed ID: 9582350

  • 1. 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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  • 2. 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
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  • 7. Mechanisms for GroEL/GroES-mediated folding of a large 86-kDa fusion polypeptide in vitro.
    Huang YS, Chuang DT.
    J Biol Chem; 1999 Apr 09; 274(15):10405-12. PubMed ID: 10187830
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  • 10. GroEL/GroES-dependent reconstitution of alpha2 beta2 tetramers of humanmitochondrial branched chain alpha-ketoacid decarboxylase. Obligatory interaction of chaperonins with an alpha beta dimeric intermediate.
    Chuang JL, Wynn RM, Song JL, Chuang DT.
    J Biol Chem; 1999 Apr 09; 274(15):10395-404. PubMed ID: 10187829
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  • 11. 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
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  • 13. 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 28; 88(3):1034-7. PubMed ID: 1885764
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  • 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
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  • 15. 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
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  • 16. 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 15; 86(1):242-7. PubMed ID: 2365818
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  • 17. 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 25; 267(18):12400-3. PubMed ID: 1352285
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  • 18. Interactions of GroEL/GroES with a heterodimeric intermediate during alpha 2beta 2 assembly of mitochondrial branched-chain alpha-ketoacid dehydrogenase. cis capping of the native-like 86-kDa intermediate by GroES.
    Song JL, Wynn RM, Chuang DT.
    J Biol Chem; 2000 Jul 21; 275(29):22305-12. PubMed ID: 10764784
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