CYP27A1
Sterol 27-hydroxylase, a mitochondrial cytochrome P-450, together with 2 protein cofactors, adrenodoxin (MIM.103260) and adrenodoxin reductase (MIM.103270), hydroxylates a variety of sterols at the C27 position.
In the bile acid synthesis pathway, sterol 27-hydroxylase catalyzes the first step in the oxidation of the side chain of sterol intermediates.
Pathology
germline mutations in cerebrotendinous xanthomatosis (MIM.213700).
See also
cholesterol metabolism
biliary acid metabolism