Literature DB >> 1121328

Thiamine increases the specific activity of human liver branched chain alpha-ketoacid dehydrogenase.

D J Danner, E D Davidson, L J Elsas.   

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Year:  1975        PMID： 1121328     DOI： 10.1038/254529a0

Source DB:  PubMed          Journal:  Nature        ISSN： 0028-0836            Impact factor:   49.962


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  9 in total

1.  Sequence of the E1 alpha subunit of branched-chain alpha-ketoacid dehydrogenase in two patients with thiamine-responsive maple syrup urine disease.

Authors:  B Zhang; R S Wappner; I K Brandt; R A Harris; D W Crabb
Journal:  Am J Hum Genet       Date:  1990-04       Impact factor: 11.025

Review 2.  Maple syrup urine disease 1954 to 1993.

Authors:  F Peinemann; D J Danner
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982

3.  Direct physical evidence for stabilization of branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.

Authors:  S C Heffelfinger; E T Sewell; L J Elsas; D J Danner
Journal:  Am J Hum Genet       Date:  1984-07       Impact factor: 11.025

4.  Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation.

Authors:  A Bodner-Leidecker; U Wendel; J M Saudubray; P Schadewaldt
Journal:  J Inherit Metab Dis       Date:  2000-12       Impact factor: 4.982

5.  Gene preference in maple syrup urine disease.

Authors:  M M Nellis; D J Danner
Journal:  Am J Hum Genet       Date:  2000-12-07       Impact factor: 11.025

6.  Branched-chain ketoacid dehydrogenase activity and growth of normal and mutant human fibroblasts: the effect of branched-chain amino acid concentration in culture medium.

Authors:  D J Danner; J H Priest
Journal:  Biochem Genet       Date:  1983-10       Impact factor: 1.890

7.  Absence of branched chain acyl-transferase as a cause of maple syrup urine disease.

Authors:  D J Danner; N Armstrong; S C Heffelfinger; E T Sewell; J H Priest; L J Elsas
Journal:  J Clin Invest       Date:  1985-03       Impact factor: 14.808

8.  Production and characterization of murine models of classic and intermediate maple syrup urine disease.

Authors:  Gregg E Homanics; Kristen Skvorak; Carolyn Ferguson; Simon Watkins; Harbhajan S Paul
Journal:  BMC Med Genet       Date:  2006-03-31       Impact factor: 2.103

9.  Genetic Predisposition to an Impaired Metabolism of the Branched-Chain Amino Acids and Risk of Type 2 Diabetes: A Mendelian Randomisation Analysis.

Authors:  Luca A Lotta; Robert A Scott; Stephen J Sharp; Stephen Burgess; Jian'an Luan; Therese Tillin; Amand F Schmidt; Fumiaki Imamura; Isobel D Stewart; John R B Perry; Luke Marney; Albert Koulman; Edward D Karoly; Nita G Forouhi; Rasmus J O Sjögren; Erik Näslund; Juleen R Zierath; Anna Krook; David B Savage; Julian L Griffin; Nishi Chaturvedi; Aroon D Hingorani; Kay-Tee Khaw; Inês Barroso; Mark I McCarthy; Stephen O'Rahilly; Nicholas J Wareham; Claudia Langenberg
Journal:  PLoS Med       Date:  2016-11-29       Impact factor: 11.069
  9 in total

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