Abstract |
Sensitive assays were developed for long chain and very long chain fatty acid oxidation in human skin fibroblast homogenates. Stearic and lignoceric acids were degraded by the fibroblasts by the beta-oxidation pathway. The cofactor requirements for stearic and lignoceric acid beta-oxidation were very similar but not identical. For example, appreciable lignoceric acid oxidation could be demonstrated only in the presence of alpha-cyclodextrin and was inhibited by Triton X-100. In Zellweger's syndrome, stearic acid beta-oxidation was partially reduced whereas lignoceric acid beta-oxidation was reduced dramatically (less than 12% activity compared to the controls). The results presented suggest that stearic acid beta-oxidation occurs in mitochondria as well as in peroxisomes, but lignoceric acid oxidation occurs entirely in the peroxisomes. We suggest that the beta-oxidation systems for stearic acid and lignoceric acid may be different.
|
Authors | H Singh, A Poulos |
Journal | Archives of biochemistry and biophysics
(Arch Biochem Biophys)
Vol. 250
Issue 1
Pg. 171-9
(Oct 1986)
ISSN: 0003-9861 [Print] United States |
PMID | 3767370
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
|
Chemical References |
- Fatty Acids
- Stearic Acids
- stearic acid
- lignoceric acid
|
Topics |
- Autoradiography
- Fatty Acids
(metabolism)
- Fibroblasts
(enzymology, metabolism)
- Humans
- Lipid Metabolism, Inborn Errors
(metabolism)
- Oxidation-Reduction
- Skin
(enzymology, metabolism)
- Stearic Acids
(metabolism)
|