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...
Topics
- Arachidonic Acid
- Arachidonic Acids
- Biological Transport, Active
- Biotin
- Carboxy-Lyases
- Cell Line
- Culture Media
- Fatty Acids
- Fibroblasts
- Humans
- Mutation
- Oleic Acid
