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[3H]biotin-labeled proteins in cultured human skin fibroblasts from patients with pyruvate carboxylase deficiency.

Abstract
Biotin containing carboxylases in cultured human skin fibroblasts were radioactively labeled by addition of [8,9-3H]biotin to biotin-depleted cell cultures. Three major bands were visualized by fluorography after sodium dodecyl sulfate-polyacrylamide gel electrophoresis of the fibroblast proteins. These bands corresponded to pyruvate carboxylase (Mr = 125,000), the biotin-containing subunit of methyl crotonyl-CoA carboxylase (Mr = 75,000) and the biotin-containing subunit of propionyl-CoA carboxylase (Mr = 73,000) as judged by molecular weight markers, purified carboxylase protein standards, and interaction with monospecific antisera. Four out of 5 cell lines from patients with classical pyruvate carboxylase deficiency (less than 5% of normal activity) labeled with this technique displayed a normal band in the position of pyruvate carboxylase while one cell line showed complete absence of any labeled protein in this area. These results demonstrate heterogeneity in the etiology of pyruvate carboxylase deficiency.
AuthorsB H Robinson, J Oei, M Saunders, R Gravel
JournalThe Journal of biological chemistry (J Biol Chem) Vol. 258 Issue 10 Pg. 6660-4 (May 25 1983) ISSN: 0021-9258 [Print] United States
PMID6406485 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Biotin
  • Carboxy-Lyases
  • Ligases
  • Carbon-Carbon Ligases
  • Pyruvate Carboxylase
  • methylcrotonoyl-CoA carboxylase
  • Methylmalonyl-CoA Decarboxylase
Topics
  • Biotin (metabolism)
  • Carbon-Carbon Ligases
  • Carboxy-Lyases (metabolism)
  • Cell Line
  • Electrophoresis, Polyacrylamide Gel
  • Fibroblasts (metabolism)
  • Humans
  • Immunosorbent Techniques
  • Ligases (metabolism)
  • Methylmalonyl-CoA Decarboxylase
  • Pyruvate Carboxylase (metabolism)
  • Pyruvate Carboxylase Deficiency Disease

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