Congenital bile acid synthesis defect

Primary bile acid biosynthesis Metabolic pathways Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol +2 more

Primary bile acid biosynthesis Steroid hormone biosynthesis Metabolic pathways Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol +3 more

Primary bile acid biosynthesis Steroid hormone biosynthesis Synthesis of bile acids and bile salts Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol Endogenous sterols Defective CYP7B1 causes Spastic paraplegia 5A, autosomal recessive (SPG5A) and Congenital bile acid synthesis defect 3 (CBAS3) +4 more

Primary bile acid biosynthesis Metabolic pathways Peroxisome Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Beta-oxidation of pristanoyl-CoA Peroxisomal protein import +4 more

ABC transporters Peroxisome ABC transporters in lipid homeostasis Class I peroxisomal membrane protein import +1 more

Primary bile acid biosynthesis Metabolic pathways PPAR signaling pathway Peroxisome Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Beta-oxidation of pristanoyl-CoA Peroxisomal protein import +4 more

ABC transporters Peroxisome ABC transporters in lipid homeostasis Class I peroxisomal membrane protein import +1 more

Primary bile acid biosynthesis Metabolic pathways Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol +2 more

Primary bile acid biosynthesis Steroid hormone biosynthesis Metabolic pathways Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol +3 more

Primary bile acid biosynthesis Steroid hormone biosynthesis Synthesis of bile acids and bile salts Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 27-hydroxycholesterol Endogenous sterols Defective CYP7B1 causes Spastic paraplegia 5A, autosomal recessive (SPG5A) and Congenital bile acid synthesis defect 3 (CBAS3) +4 more

Primary bile acid biosynthesis Metabolic pathways Peroxisome Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol Synthesis of bile acids and bile salts via 24-hydroxycholesterol Beta-oxidation of pristanoyl-CoA Peroxisomal protein import +4 more