Article
Fatty acid transport in multiple carboxylase deficiency fibroblasts.
Journal of inherited metabolic disease - 1 Jan 1990
Packman S, Whitney S
Abstract excerpt
Holocarboxylase synthetase (HS) and biotinidase deficiencies have been identified as causes of biotin-responsive multiple carboxylase deficiency. Acetyl-CoA carboxylase (ACC) deficiency has been shown to occur in multiple carboxylase deficiency, and HS(-) fibroblasts are being employed to investigate compensatory regulatory responses in cells deficient in ACC. In previous studies, biotin starved HS(-) fibroblasts...
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