Article
Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency: does lipoic acid have a protective role?
Pediatric research - 1 Oct 2020
Nochi Zahra, Birkler Rune Isak Dupont, Fernandez-Guerra Paula, Hansen Jakob, Wibrand Flemming, Corydon Thomas Juhl, Gregersen Niels, Olsen Rikke Katrine Jentoft
Abstract excerpt
BACKGROUND: Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD) is the most frequent fatty acid oxidation (FAO) defect in humans. MCAD-deficient fibroblasts are more resistant to oxidative stress-induced cell death than other FAO defects and healthy controls. METHODS: Herein we investigate the antioxidant response and mitochondrial function in fibroblasts from MCAD-deficient patients (c.985 A>G/c.985...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
