119 related articles for article (PubMed ID: 2699394)
1. Molecular studies of mammalian branched-chain alpha-keto acid dehydrogenase complexes: domain structures, expression, and inborn errors.
Chuang DT
Ann N Y Acad Sci; 1989; 573():137-54. PubMed ID: 2699394
[TBL] [Abstract][Full Text] [Related]
2. Conservation of primary structure in the lipoyl-bearing and dihydrolipoyl dehydrogenase binding domains of mammalian branched-chain alpha-keto acid dehydrogenase complex: molecular cloning of human and bovine transacylase (E2) cDNAs.
Lau KS; Griffin TA; Hu CW; Chuang DT
Biochemistry; 1988 Mar; 27(6):1972-81. PubMed ID: 2837277
[TBL] [Abstract][Full Text] [Related]
3. Characterization and conservation of the inner E2 core domain structure of branched-chain alpha-keto acid dehydrogenase complex from bovine liver. Construction of a cDNA encoding the entire transacylase (E2b) precursor.
Griffin TA; Lau KS; Chuang DT
J Biol Chem; 1988 Oct; 263(28):14008-14. PubMed ID: 3049570
[TBL] [Abstract][Full Text] [Related]
4. 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]
5. cDNA cloning of the chicken branched-chain alpha-keto acid dehydrogenase complex. Chicken-specific residues of the acyltransferase affect the overall activity and the interaction with the dehydrogenase.
Ono K; Hakozaki M; Suzuki T; Mori T; Hata H; Kochi H
Eur J Biochem; 2001 Feb; 268(3):727-36. PubMed ID: 11168412
[TBL] [Abstract][Full Text] [Related]
6. Complete primary structure of the transacylase (E2b) subunit of the human branched chain alpha-keto acid dehydrogenase complex.
Nobukuni Y; Mitsubuchi H; Endo F; Matsuda I
Biochem Biophys Res Commun; 1989 Jun; 161(3):1035-41. PubMed ID: 2742576
[TBL] [Abstract][Full Text] [Related]
7. Genetic reconstruction and characterization of the recombinant transacylase (E2b) component of bovine branched-chain alpha-keto acid dehydrogenase complex. Implication of histidine 391 as an active site residue.
Griffin TA; Chuang DT
J Biol Chem; 1990 Aug; 265(22):13174-80. PubMed ID: 2198287
[TBL] [Abstract][Full Text] [Related]
8. Expression and assembly of mature apotransacylase (E2b) of bovine branched-chain alpha-keto acid dehydrogenase complex in Escherichia coli. Demonstration of transacylase activity and modification by lipoylation.
Griffin TA; Wynn RM; Chuang DT
J Biol Chem; 1990 Jul; 265(20):12104-10. PubMed ID: 2195031
[TBL] [Abstract][Full Text] [Related]
9. 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]
10. 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]
11. 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]
12. Subunit structure of the dihydrolipoyl transacylase component of branched-chain alpha-keto acid dehydrogenase complex from bovine liver. Mapping of the lipoyl-bearing domain by limited proteolysis.
Hu CW; Griffin TA; Lau KS; Cox RP; Chuang DT
J Biol Chem; 1986 Jan; 261(1):343-9. PubMed ID: 2416750
[TBL] [Abstract][Full Text] [Related]
13. 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]
14. Maple syrup urine disease caused by a partial deletion in the inner E2 core domain of the branched chain alpha-keto acid dehydrogenase complex due to aberrant splicing. A single base deletion at a 5'-splice donor site of an intron of the E2 gene disrupts the consensus sequence in this region.
Mitsubuchi H; Nobukuni Y; Akaboshi I; Indo Y; Endo F; Matsuda I
J Clin Invest; 1991 Apr; 87(4):1207-11. PubMed ID: 2010537
[TBL] [Abstract][Full Text] [Related]
15. Comparison of the amino acid sequences of the transacylase components of branched chain oxoacid dehydrogenase of Pseudomonas putida, and the pyruvate and 2-oxoglutarate dehydrogenases of Escherichia coli.
Burns G; Brown T; Hatter K; Sokatch JR
Eur J Biochem; 1988 Sep; 176(1):165-9. PubMed ID: 3046941
[TBL] [Abstract][Full Text] [Related]
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; 86(1):242-7. PubMed ID: 2365818
[TBL] [Abstract][Full Text] [Related]
17. 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]
18. 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]
19. 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]
20. Molecular cloning of the mature E1b-beta subunit of human branched-chain alpha-keto acid dehydrogenase complex.
Chuang JL; Cox RP; Chuang DT
FEBS Lett; 1990 Mar; 262(2):305-9. PubMed ID: 2335211
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
