LMPD Database

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LMP002471

UniProt Annotations

Entry Information
Gene Namecytochrome P450, family 27, subfamily A, polypeptide 1
Protein EntryCP27A_HUMAN
UniProt IDQ02318
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity5-beta-cholestane-3-alpha,7-alpha,12-alpha- triol + 3 NADPH + 3 O(2) = (25R)-3-alpha,7-alpha,12-alpha- trihydroxy-5-beta-cholestan-26-oate + 3 NADP(+) + 4 H(2)O.
CofactorName=heme; Xref=ChEBI
DiseaseCerebrotendinous xanthomatosis (CTX) [MIM
FunctionCatalyzes the first step in the oxidation of the side chain of sterol intermediates; the 27-hydroxylation of 5-beta- cholestane-3-alpha,7-alpha,12-alpha-triol. Has also a vitamin D3- 25-hydroxylase activity.
PathwayHormone biosynthesis; cholecalciferol biosynthesis.
SimilarityBelongs to the cytochrome P450 family.
Subcellular LocationMitochondrion membrane.