mitochondrial trifunctional protein deficiency

Fatty acid elongation Fatty acid degradation Valine, leucine and isoleucine degradation Metabolic pathways Fatty acid metabolism Acyl chain remodeling of CL Beta oxidation of myristoyl-CoA to lauroyl-CoA mitochondrial fatty acid beta-oxidation of unsaturated fatty acids Beta oxidation of palmitoyl-CoA to myristoyl-CoA Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA Beta oxidation of octanoyl-CoA to hexanoyl-CoA Beta oxidation of hexanoyl-CoA to butanoyl-CoA +10 more