Long Chain fatty Acids Analysis in Egyptian Patients with Inherited Peroxisomal Disorders
VLCFA
Peroxisomal disorders are a group of genetically heterogeneous metabolic diseases that share dysfunction of peroxisomes. More than 50 enzymes have been discovered in mammalian peroxisomes that participate in various metabolic pathways. β-oxidation of very long chain fatty acid (VLCFA) is one of the most important functions of the peroxisome. Peroxisomes work in conjunction with mitochondria to shorten the VLCFA by β-oxidation, which are in turn degraded to completion in mitochondria. The end result is formation of acetyl-CoA units, which are degraded in the Krebs cycle to produce energy (ATP).