Names & Taxonomy

Uniprot ID:
Q02318
Entry Name:
CP27A_HUMAN
Status:
reviewed
Protein Names:
Sterol 26-hydroxylase, mitochondrial (EC 1.14.15.15) (5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase) (Cytochrome P-450C27/25) (Cytochrome P450 27) (Sterol 27-hydroxylase) (Vitamin D(3) 25-hydroxylase)
Gene Names:
CYP27A1 CYP27
Gene Names Primary:
CYP27A1
Organism:
Homo sapiens (Human)

Structure

Length:
531
Sequence:
MAALGCARLRWALRGAGRGLCPHGARAKAAIPAALPSDKATGAPGAGPGVRRRQRSLEEIPRLGQLRFFFQLFVQGYALQLHQLQVLYKAKYGPMWMSYLGPQMHVNLASAPLLEQVMRQEGKYPVRNDMELWKEHRDQHDLTYGPFTTEGHHWYQLRQALNQRLLKPAEAALYTDAFNEVIDDFMTRLDQLRAESASGNQVSDMAQLFYYFALEAICYILFEKRIGCLQRSIPEDTVTFVRSIGLMFQNSLYATFLPKWTRPVLPFWKRYLDGWNAIFSFGKKLIDEKLEDMEAQLQAAGPDGIQVSGYLHFLLASGQLSPREAMGSLPELLMAGVDTTSNTLTWALYHLSKDPEIQEALHEEVVGVVPAGQVPQHKDFAHMPLLKAVLKETLRLYPVVPTNSRIIEKEIEVDGFLFPKNTQFVFCHYVVSRDPTAFSEPESFQPHRWLRNSQPATPRIQHPFGSVPFGYGVRACLGRRIAELEMQLLLARLIQKYKVVLAPETGELKSVARIVLVPNKKVGLQFLQRQC
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion membrane.

Function

Function:
Catalyzes 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.
Pathway:
Hormone biosynthesis; cholecalciferol biosynthesis.
Catalytic Activity:
5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol + 6 reduced adrenodoxin + 6 H(+) + 3 O(2) = (25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestan-26-oate + 6 oxidized adrenodoxin + 4 H(2)O.
Cofactor:
COFACTOR: Name=heme; Xref=ChEBI:CHEBI:30413;
Cross Reference Drug Bank:
DB06777 DB00169 DB06410 DB00153 DB00082
Gene Ontology Go:
mitochondrial inner membrane
mitochondrial matrix
cholestanetriol 26-monooxygenase activity
heme binding
iron ion binding
steroid hydroxylase activity
bile acid biosynthetic process
bile acid metabolic process
small molecule metabolic process
sterol metabolic process
xenobiotic metabolic process
Gene Ontology Biological Process:
bile acid biosynthetic process
bile acid metabolic process
small molecule metabolic process
sterol metabolic process
xenobiotic metabolic process
Gene Ontology Molecular Function:
cholestanetriol 26-monooxygenase activity
heme binding
iron ion binding
steroid hydroxylase activity
Gene Ontology Cellular Component:
mitochondrial inner membrane
mitochondrial matrix
Keywords:
3D-structure
Acetylation
Cataract
Complete proteome
Disease mutation
Heme
Iron
Membrane
Metal-binding
Mitochondrion
Monooxygenase
NADP
Oxidoreductase
Polymorphism
Reference proteome
Transit peptide

Publication

PubMed ID:
1708392 7690968 14702039 15489334 8514861 24275569 2019602 7915755 9186905 9790667 12000359