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7. Maple syrup urine disease in Poll Shorthorn calves. Healy PJ; Dennis JA; Harper PA; Graham R; Reuter RE Aust Vet J; 1992 Jun; 69(6):143-4. PubMed ID: 1642599 [No Abstract] [Full Text] [Related]
8. Maple syrup urine disease: clinical and biochemical significance of gene analysis. Nobukuni Y; Mitsubuchi H; Akaboshi I; Indo Y; Endo F; Matsuda I J Inherit Metab Dis; 1991; 14(5):787-92. PubMed ID: 1664011 [No Abstract] [Full Text] [Related]
9. 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 Am J Hum Genet; 1990 Apr; 46(4):843-6. PubMed ID: 2316528 [TBL] [Abstract][Full Text] [Related]
10. A distinct variant of intermediate maple syrup urine disease. Gonzalez-Rios MC; Chuang DT; Cox RP; Schmidt K; Knopf K; Packman S Clin Genet; 1985 Feb; 27(2):153-9. PubMed ID: 3978850 [TBL] [Abstract][Full Text] [Related]
11. Complementation analysis in lymphoid cells from five patients with different forms of maple syrup urine disease. Jinno Y; Akaboshi I; Matsuda I Hum Genet; 1984; 68(1):54-6. PubMed ID: 6500555 [TBL] [Abstract][Full Text] [Related]
12. Genetic defects in E3 component of alpha-keto acid dehydrogenase complexes. Patel MS; Hong YS; Kerr DS Methods Enzymol; 2000; 324():453-64. PubMed ID: 10989452 [No Abstract] [Full Text] [Related]
13. Targeting E3 component of alpha-keto acid dehydrogenase complexes. Johnson MT; Yang HS; Patel MS Methods Enzymol; 2000; 324():465-76. PubMed ID: 10989453 [No Abstract] [Full Text] [Related]
14. A nonsense mutation (R242X) in the branched-chain alpha-keto acid dehydrogenase E1alpha subunit gene (BCKDHA) as a cause of maple syrup urine disease. Mutations in brief no. 160. Online. Chinsky J; Appel M; Almashanu S; Costeas P; Ambulos N; Carmi R Hum Mutat; 1998; 12(2):136. PubMed ID: 10694918 [TBL] [Abstract][Full Text] [Related]
15. 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]
16. Evidence for both a regulatory mutation and a structural mutation in a family with maple syrup urine disease. Zhang B; Edenberg HJ; Crabb DW; Harris RA J Clin Invest; 1989 Apr; 83(4):1425-9. PubMed ID: 2703538 [TBL] [Abstract][Full Text] [Related]
17. Absence of branched chain acyl-transferase as a cause of maple syrup urine disease. Danner DJ; Armstrong N; Heffelfinger SC; Sewell ET; Priest JH; Elsas LJ J Clin Invest; 1985 Mar; 75(3):858-60. PubMed ID: 3980729 [TBL] [Abstract][Full Text] [Related]
18. 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]
19. Maple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E1beta mRNAs arises from intronic sequences. Chuang JL; Cox RP; Chuang DT Am J Hum Genet; 1996 Jun; 58(6):1373-7. PubMed ID: 8651316 [No Abstract] [Full Text] [Related]