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25. 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]
26. Molecular genetic characterization of maple syrup urine disease in European families. Peinemann F; Wendel U; Danner DJ Biochem Med Metab Biol; 1993 Dec; 50(3):338-45. PubMed ID: 8123297 [TBL] [Abstract][Full Text] [Related]
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28. 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]
29. Maple syrup urine disease as a cause of spongiform encephalopathy in calves. Harper PA; Healy PJ; Dennis JA Vet Rec; 1986 Jul; 119(3):62-5. PubMed ID: 3529596 [TBL] [Abstract][Full Text] [Related]
30. 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]
31. 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]
32. 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]
33. Gene preference in maple syrup urine disease. Nellis MM; Danner DJ Am J Hum Genet; 2001 Jan; 68(1):232-7. PubMed ID: 11112664 [TBL] [Abstract][Full Text] [Related]
34. Molecular basis of intermittent maple syrup urine disease: novel mutations in the E2 gene of the branched-chain alpha-keto acid dehydrogenase complex. Tsuruta M; Mitsubuchi H; Mardy S; Miura Y; Hayashida Y; Kinugasa A; Ishitsu T; Matsuda I; Indo Y J Hum Genet; 1998; 43(2):91-100. PubMed ID: 9621512 [TBL] [Abstract][Full Text] [Related]
35. Molecular defects in the E1 alpha subunit of the branched-chain alpha-ketoacid dehydrogenase complex that cause maple syrup urine disease. Zhang B; Zhao Y; Harris RA; Crabb DW Mol Biol Med; 1991 Feb; 8(1):39-47. PubMed ID: 1943689 [TBL] [Abstract][Full Text] [Related]
36. Stable correction of maple syrup urine disease in cells from a Mennonite patient by retroviral-mediated gene transfer. Koyata H; Cox RP; Chuang DT Biochem J; 1993 Nov; 295 ( Pt 3)(Pt 3):635-9. PubMed ID: 8240269 [TBL] [Abstract][Full Text] [Related]
37. Occurrence of a Tyr393----Asn (Y393N) mutation in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients from a Mennonite population. Fisher CR; Fisher CW; Chuang DT; Cox RP Am J Hum Genet; 1991 Aug; 49(2):429-34. PubMed ID: 1867199 [TBL] [Abstract][Full Text] [Related]
38. Occurrence of a 2-bp (AT) deletion allele and a nonsense (G-to-T) mutant allele at the E2 (DBT) locus of six patients with maple syrup urine disease: multiple-exon skipping as a secondary effect of the mutations. Fisher CW; Fisher CR; Chuang JL; Lau KS; Chuang DT; Cox RP Am J Hum Genet; 1993 Feb; 52(2):414-24. PubMed ID: 8430702 [TBL] [Abstract][Full Text] [Related]
39. Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population. Edelmann L; Wasserstein MP; Kornreich R; Sansaricq C; Snyderman SE; Diaz GA Am J Hum Genet; 2001 Oct; 69(4):863-8. PubMed ID: 11509994 [TBL] [Abstract][Full Text] [Related]
40. cDNA cloning of the E1 alpha subunit of the branched-chain alpha-keto acid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease. Zhang B; Kuntz MJ; Goodwin GW; Edenberg HJ; Crabb DW; Harris RA Ann N Y Acad Sci; 1989; 573():130-6. PubMed ID: 2634344 [TBL] [Abstract][Full Text] [Related] [Previous] [Next] [New Search]