The involvement of TFP in these functions suggests that OXPHOS dysfunction could play a role in the pathogenesis of generalized HADHA TFP deficiency (OMIM #609015), generalized HADHB TFP deficiency (OMIM #620300), and isolated LCHAD deficiency (OMIM #609016), especially the development of retinopathy, peripheral neuropathy, and cardiomyopathy. The gene discussed is HADHB; the disease is mitochondrial trifunctional protein deficiency.