... situations, long- chain fatty acids cannot be oxidized, and accumulate in tissues as long- chain acyl-CoAs and acylcarnitines[5]. However, despite the well-known mechanism of long- chain acylcarnitine ... VLCAD KO mice S. Tucci et al.4704 FEBS Journal 277 (2010) 4699–4708 ª 2010 The Authors Journal compilation ª 2010 FEBS Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient ... observed in human VLCADD [5]. In fact, in both mice and humans, clinical symptoms becomemainly evident as a consequence of triggers suchas fasting, resulting in the accumulation of long- chain acylcarnitines,...