Bifunctional enzyme deficiency

Fatty acid degradation Valine, leucine and isoleucine degradation Lysine degradation Tryptophan metabolism beta-Alanine metabolism Propanoate metabolism Butanoate metabolism Metabolic pathways Fatty acid metabolism PPAR signaling pathway Peroxisome Beta-oxidation of very long chain fatty acids Peroxisomal protein import +10 more

Primary bile acid biosynthesis Biosynthesis of unsaturated fatty acids Metabolic pathways Fatty acid metabolism Peroxisome Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol alpha-linolenic acid (ALA) metabolism Beta-oxidation of pristanoyl-CoA Beta-oxidation of very long chain fatty acids Peroxisomal protein import +7 more

Primary bile acid biosynthesis Biosynthesis of unsaturated fatty acids Metabolic pathways Fatty acid metabolism Peroxisome Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol alpha-linolenic acid (ALA) metabolism Beta-oxidation of pristanoyl-CoA Beta-oxidation of very long chain fatty acids Peroxisomal protein import +7 more