New hope in treating progressive familial intrahepatic cholestasis in children

Figure 1 Bile acid synthesis pathways. CYP7A1: Cytochrome P450 Family 7 Subfamily A Member 1 (Cholesterol 7α-hydroxylase; rate-limiting enzyme in the classic bile acid synthesis pathway); CYP8B1: Cytochrome P450 Family 8 Subfamily B Member 1 (Also called sterol 12α-hydroxylase; determines the ratio of cholic acid to chenodeoxycholic acid); CYP27A1: Cytochrome P450 Family 27 Subfamily A Member 1 (Sterol 27-hydroxylase; important for the alternative bile acid synthesis pathway); CYP7B1: Cytochrome P450 Family 7 Subfamily B Member 1 (Oxysterol 7α-hydroxylase; involved in the alternative pathway of bile acid synthesis); BACS: Bile Acid-Coenzyme A Synthase (Enzyme involved in bile acid conjugation); BAAT: Bile Acid-CoA: Amino Acid N-Acyltransferase (Conjugates bile acids with glycine or taurine); CA: Cholic Acid (Primary bile acid synthesized via the classic pathway); CDCA: Chenodeoxycholic Acid (Primary bile acid synthesized mainly via the alternative pathway).