Literature DB >> 1167168

Subcellular distribution and cofactor function of human branched chain alpha-ketoacid dehydrogenase in normal and mutant cultured skin fibroblasts.

D J Danner, L J Elsas.   

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Year:  1975        PMID： 1167168     DOI： 10.1016/0006-2944(75)90135-0

Source DB:  PubMed          Journal:  Biochem Med        ISSN： 0006-2944


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  6 in total

1.  Maple syrup urine disease: branched-chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids.

Authors:  J Dancis; J Hutzler; R P Cox
Journal:  Am J Hum Genet       Date:  1977-05       Impact factor: 11.025

2.  Altered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syrup urine disease.

Authors:  Y Indo; A Kitano; F Endo; I Akaboshi; I Matsuda
Journal:  J Clin Invest       Date:  1987-07       Impact factor: 14.808

3.  Detection of heterozygotes in maple-syrup-urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures.

Authors:  D T Chuang; L S Ku; D S Kerr; R P Cox
Journal:  Am J Hum Genet       Date:  1982-05       Impact factor: 11.025

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

6.  Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects.

Authors:  D T Chuang; W L Niu; R P Cox
Journal:  Biochem J       Date:  1981-10-15       Impact factor: 3.857
  6 in total

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