Similar articles for PMID: 2316528

Year	Number of Results
1978	1
1981	1
1982	4
1983	1
1984	2
1985	6
1987	3
1988	7
1989	6
1990	8
1991	13
1992	7
1993	7
1994	5
1995	4
1996	2
1997	3
1998	5
1999	2
2000	8
2001	7
2002	5
2004	1
2006	1
2009	1
2010	1
2011	1
2012	4
2013	1
2014	2
2022	1
2023	1
2024	1

1

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. Zhang B, et al. Am J Hum Genet. 1990 Apr;46(4):843-6. Am J Hum Genet. 1990. PMID: 2316528 Free PMC article.

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. Harris RA, et al. Adv Enzyme Regul. 1990;30:245-63. doi: 10.1016/0065-2571(90)90021-s. Adv Enzyme Regul. 1990. PMID: 2403034

3

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. Hu CW, et al. J Biol Chem. 1988 Jun 25;263(18):9007-14. J Biol Chem. 1988. PMID: 3379058 Free article.

4

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. Wynn RM, et al. J Biol Chem. 1998 May 22;273(21):13110-8. doi: 10.1074/jbc.273.21.13110. J Biol Chem. 1998. PMID: 9582350 Free article.

5

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. Nobukuni Y, et al. J Clin Invest. 1990 Jul;86(1):242-7. doi: 10.1172/JCI114690. J Clin Invest. 1990. PMID: 2365818 Free PMC article.

6

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. Nobukuni Y, et al. J Clin Invest. 1991 May;87(5):1862-6. doi: 10.1172/JCI115209. J Clin Invest. 1991. PMID: 2022752 Free PMC article.

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. Zhang B, et al. J Biol Chem. 1990 Feb 15;265(5):2425-7. J Biol Chem. 1990. PMID: 2303405 Free article.

8

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. Fisher CW, et al. J Biol Chem. 1989 Feb 25;264(6):3448-53. J Biol Chem. 1989. PMID: 2914958 Free article.

9

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. Matsuda I, et al. Biochem Biophys Res Commun. 1990 Oct 30;172(2):646-51. doi: 10.1016/0006-291x(90)90723-z. Biochem Biophys Res Commun. 1990. PMID: 2241958

10

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. Chuang JL, et al. J Clin Invest. 1995 Mar;95(3):954-63. doi: 10.1172/JCI117804. J Clin Invest. 1995. PMID: 7883996 Free PMC article.

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