192 related articles for article (PubMed ID: 6661177)
1. 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; 21(9-10):895-905. PubMed ID: 6661177
[TBL] [Abstract][Full Text] [Related]
2. 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]
3. Primary human fibroblasts from a maple syrup urine disease patient undergo apoptosis following exposure to physiological concentrations of branched chain amino acids.
Jouvet P; Kozma M; Mehmet H
Ann N Y Acad Sci; 2000; 926():116-21. PubMed ID: 11193026
[TBL] [Abstract][Full Text] [Related]
4. Metabolism of branched-chain amino acids in fibroblasts from patients with maple syrup urine disease and other abnormalities of branched-chain ketoacid dehydrogenase activity.
Yoshida I; Sweetman L; Nyhan WL
Pediatr Res; 1986 Feb; 20(2):169-74. PubMed ID: 3080729
[TBL] [Abstract][Full Text] [Related]
5. Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.
Wynn RM; Ho R; Chuang JL; Chuang DT
J Biol Chem; 2001 Feb; 276(6):4168-74. PubMed ID: 11069910
[TBL] [Abstract][Full Text] [Related]
6. Reversion of the maple syrup urine disease phenotype of impaired branched chain alpha-ketoacid dehydrogenase complex activity in fibroblasts from an affected child.
Litwer S; Herring WJ; Danner DJ
J Biol Chem; 1989 Sep; 264(25):14597-600. PubMed ID: 2768232
[TBL] [Abstract][Full Text] [Related]
7. 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]
8. Analysis of maple syrup urine disease in cell culture: use of substrates.
Schadewaldt P; Beck K; Wendel U
Clin Chim Acta; 1989 Sep; 184(1):47-56. PubMed ID: 2598467
[TBL] [Abstract][Full Text] [Related]
9. 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; 276(43):40241-6. PubMed ID: 11507102
[TBL] [Abstract][Full Text] [Related]
10. 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; 200(1):59-67. PubMed ID: 6895847
[TBL] [Abstract][Full Text] [Related]
11. 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; 147(2):103-8. PubMed ID: 3987057
[TBL] [Abstract][Full Text] [Related]
12. 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]
13. Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells.
Nellis MM; Doering CB; Kasinski A; Danner DJ
Am J Physiol Endocrinol Metab; 2002 Oct; 283(4):E853-60. PubMed ID: 12217904
[TBL] [Abstract][Full Text] [Related]
14. Controlled overexpression of BCKD kinase expression: metabolic engineering applied to BCAA metabolism in a mammalian system.
Doering CB; Williams IR; Danner DJ
Metab Eng; 2000 Oct; 2(4):349-56. PubMed ID: 11120646
[TBL] [Abstract][Full Text] [Related]
15. Diagnosis and treatment of maple syrup disease: a study of 36 patients.
Morton DH; Strauss KA; Robinson DL; Puffenberger EG; Kelley RI
Pediatrics; 2002 Jun; 109(6):999-1008. PubMed ID: 12042535
[TBL] [Abstract][Full Text] [Related]
16. [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]
17. 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]
18. Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis.
Turchany JM; Leung PS; Iwayama T; Jefferson DM; Ishida J; Yamaguchi M; Munoz S; Danner DJ; Dickson ER; Gershwin ME
J Autoimmun; 1993 Aug; 6(4):459-66. PubMed ID: 8216688
[TBL] [Abstract][Full Text] [Related]
19. Maple syrup urine disease: it has come a long way.
Chuang DT
J Pediatr; 1998 Mar; 132(3 Pt 2):S17-23. PubMed ID: 9546032
[TBL] [Abstract][Full Text] [Related]
20. Thiamine response in maple syrup urine disease.
Fernhoff PM; Lubitz D; Danner DJ; Dembure PP; Schwartz HP; Hillman R; Bier DM; Elsas LJ
Pediatr Res; 1985 Oct; 19(10):1011-6. PubMed ID: 3903643
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
