Very long chain fatty acids activate NADPH oxidase in human dermal fibroblasts.

Very long chain fatty acids (VLCFAs) are exclusively oxidized in peroxisomes and their levels are significantly increased in tissues of patients with peroxisomal disorders. Although the biochemical indicators of peroxisomal dysfunction, such as elevated VLCFAs, are well known, the mechanisms of pathogenesis of peroxisomal diseases are unclear. In this study we have examined the effect of VLCFAs on NADPH oxidase (NOX), a complex enzyme system responsible for the production of superoxide anions, in order to understand the oxidative stress-mediated mechanisms involved in pathology of peroxisomal disorders.

Carnitine prevents cyclic GMP-induced inhibition of peroxisomal enzyme activities.

Peroxisomes, also termed as microbodies, are now known to carry out several specialized metabolic activities that are vital to cellular function. A defect in peroxisomal function leads to development of a fatal human disease, and a number of peroxisomal disorders are now linked to inherited peroxisomal enzyme abnormalities. Peroxisomal enzyme activities are also altered during pathophysiological conditions through various endogenously produced bio-molecules such as nitric oxide (NO).