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4. The role of thiamin in maple syrup urine disease. Elsas LJ; Danner DJ Ann N Y Acad Sci; 1982; 378():404-21. PubMed ID: 7044230 [No Abstract] [Full Text] [Related]
5. Diabetes mellitus, thiamine-dependent megaloblastic anemia, and sensorineural deafness associated with deficient alpha-ketoglutarate dehydrogenase activity. Abboud MR; Alexander D; Najjar SS J Pediatr; 1985 Oct; 107(4):537-41. PubMed ID: 4045602 [TBL] [Abstract][Full Text] [Related]
6. Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate. Chuang DT; Ku LS; Cox RP Proc Natl Acad Sci U S A; 1982 May; 79(10):3300-4. PubMed ID: 6954481 [TBL] [Abstract][Full Text] [Related]
7. E2 transacylase-deficient (type II) maple syrup urine disease. Aberrant splicing of E2 mRNA caused by internal intronic deletions and association with thiamine-responsive phenotype. Chuang JL; Cox RP; Chuang DT J Clin Invest; 1997 Aug; 100(3):736-44. PubMed ID: 9239422 [TBL] [Abstract][Full Text] [Related]
8. Biochemical basis of thiamin-responsive maple syrup urine disease. Chuang DT; Ku LS; Cox RP Trans Assoc Am Physicians; 1982; 95():196-204. PubMed ID: 7182976 [TBL] [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]
11. 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]
12. 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]
13. Interrelation between the metabolism of L-isoleucine and L-allo-isoleucine in patients with maple syrup urine disease. Wendel U; Langenbeck U; Seakins JW Pediatr Res; 1989 Jan; 25(1):11-4. PubMed ID: 2919111 [TBL] [Abstract][Full Text] [Related]
14. Detection of heterozygotes in maple-syrup-urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures. Chuang DT; Ku LS; Kerr DS; Cox RP Am J Hum Genet; 1982 May; 34(3):416-24. PubMed ID: 7081220 [TBL] [Abstract][Full Text] [Related]
15. MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation. Jan W; Zimmerman RA; Wang ZJ; Berry GT; Kaplan PB; Kaye EM Neuroradiology; 2003 Jun; 45(6):393-9. PubMed ID: 12736767 [TBL] [Abstract][Full Text] [Related]
17. Thiamine-responsive megaloblastic anemia, sensorineural deafness, and diabetes mellitus: A new syndrome? Viana MB; Carvalho RI J Pediatr; 1978 Aug; 93(2):235-8. PubMed ID: 671156 [TBL] [Abstract][Full Text] [Related]
18. 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]
19. 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]
20. 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] [Next] [New Search]