Mechanistic Bases of Neurotoxicity Provoked by Fatty Acids Accumulating in MCAD and LCHAD Deficiencies

Fatty acid oxidation defects (FAODs) are inherited metabolic disorders caused by deficiency of specific enzyme activities or transport proteins involved in the mitochondrial catabolism of fatty acids. Medium-chain fatty acyl-CoA dehydrogenase (MCAD) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LC...

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Bibliographic Details
Main Authors: Alexandre U. Amaral PhD, Cristiane Cecatto MSc, Janaína C. da Silva PhD, Alessandro Wajner PhD, Moacir Wajner MD, PhD
Format: Article
Language:English
Published: SciELO 2017-03-01
Series:Journal of Inborn Errors of Metabolism and Screening
Online Access:https://doi.org/10.1177/2326409817701472