Literature DB >> 7396533

Stabilization of mammalian liver branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.

D J Danner, S K Lemmon, L J Elsas.   

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Year:  1980        PMID： 7396533     DOI： 10.1016/0003-9861(80)90401-4

Source DB:  PubMed          Journal:  Arch Biochem Biophys        ISSN： 0003-9861            Impact factor:   4.013


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  5 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

2.  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

3.  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.

Authors:  J L Chuang; R P Cox; D T Chuang
Journal:  J Clin Invest       Date:  1997-08-01       Impact factor: 14.808

4.  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

5.  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
  5 in total

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