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CYP51A1

Function

Sterol 14alpha-demethylase that plays a critical role in the cholesterol biosynthesis pathway, being cholesterol the major sterol component in mammalian membranes as well as a precursor for bile acid and steroid hormone synthesis (PubMed:20149798, PubMed:8619637, PubMed:9559662). Cytochrome P450 monooxygenase that catalyzes the three-step oxidative removal of the 14alpha-methyl group (C-32) of sterols such as lanosterol (lanosta-8,24-dien-3beta-ol) and 24,25-dihydrolanosterol (DHL) in the form of formate, and converts the sterols to 4,4-dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol and 4,4-dimethyl-8,14-cholestadien-3beta-ol, respectively, which are intermediates of cholesterol biosynthesis (PubMed:20149798, PubMed:8619637, PubMed:9559662). Can also demethylate substrates not intrinsic to mammals, such as eburicol (24-methylene-24,25-dihydrolanosterol), but at a lower rate than DHL (PubMed:9559662).

Pathway

Steroid biosynthesis; zymosterol biosynthesis; zymosterol from lanosterol: step 1/6.

Sequence Similarities

Belongs to the cytochrome P450 family.

Tissue Specificity

Ubiquitously expressed with highest levels in testis, ovary, adrenal, prostate, liver, kidney and lung.

Cellular localization

Alternative names

CYP51, CYP51A1, Lanosterol 14-alpha demethylase, LDM, CYPLI, Cytochrome P450 51A1, Cytochrome P450-14DM, Cytochrome P450LI, Sterol 14-alpha demethylase, Cytochrome P45014DM

swissprot:Q16850 omim:601637 entrezGene:1595