Article
Defective oxidation of pristanic acid by fibroblasts from patients with disorders in propionic acid metabolism.
Clinical genetics - 1 Feb 1990
Poulos A, Johnson D, Singh H
Abstract excerpt
The alpha-methyl fatty acid, pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) is oxidised rapidly by normal skin fibroblasts in culture to CO2 and to water-soluble metabolites. The latter are secreted into the culture medium. Fibroblasts from patients with propionyl CoA carboxylase, an...
Topics
- Amino Acid Metabolism, Inborn Errors
- Cell Line
- Fatty Acids
- Fibroblasts
- Humans
- Mutation
- Oxidation-Reduction
- Propionates