General Information of the Protein
Protein ID
PT05883
Protein Name
Sterol 26-hydroxylase, mitochondrial
Secondarily
Protein Name
5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 26-hydroxylase
Cytochrome P-450C27/25
Cytochrome P450 27
Sterol 27-hydroxylase
Vitamin D(3) 25-hydroxylase
Gene Name
CYP27A1
Secondarily
Gene Name
CYP27
Sequence
MAALGCARLRWALRGAGRGLCPHGARAKAAIPAALPSDKATGAPGAGPGVRRRQRSLEEIPRLGQLRFFFQLFVQGYALQLHQLQVLYKAKYGPMWMSYLGPQMHVNLASAPLLEQVMRQEGKYPVRNDMELWKEHRDQHDLTYGPFTTEGHHWYQLRQALNQRLLKPAEAALYTDAFNEVIDDFMTRLDQLRAESASGNQVSDMAQLFYYFALEAICYILFEKRIGCLQRSIPEDTVTFVRSIGLMFQNSLYATFLPKWTRPVLPFWKRYLDGWNAIFSFGKKLIDEKLEDMEAQLQAAGPDGIQVSGYLHFLLASGQLSPREAMGSLPELLMAGVDTTSNTLTWALYHLSKDPEIQEALHEEVVGVVPAGQVPQHKDFAHMPLLKAVLKETLRLYPVVPTNSRIIEKEIEVDGFLFPKNTQFVFCHYVVSRDPTAFSEPESFQPHRWLRNSQPATPRIQHPFGSVPFGYGVRACLGRRIAELEMQLLLARLIQKYKVVLAPETGELKSVARIVLVPNKKVGLQFLQRQC
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Organism
Homo sapiens, Human
Protein Classification
Enzyme
>
Cytochrome P450
>
Cytochrome P450 family 27
>
Cytochrome P450 family 27A
>
Cytochrome P450 27A1
Function
Cytochrome P450 monooxygenase that catalyzes regio- and stereospecific hydroxylation of cholesterol and its derivatives. Hydroxylates (with R stereochemistry) the terminal methyl group of cholesterol side-chain in a three step reaction to yield at first a C26 alcohol, then a C26 aldehyde and finally a C26 acid (PubMed:9660774, PubMed:12077124, PubMed:21411718, PubMed:28190002). Regulates cholesterol homeostasis by catalyzing the conversion of excess cholesterol to bile acids via both the 'neutral' (classic) and the 'acid' (alternative) pathways (PubMed:9660774, PubMed:1708392, PubMed:11412116, PubMed:2019602, PubMed:7915755, PubMed:9186905, PubMed:9790667). May also regulate cholesterol homeostasis via generation of active oxysterols, which act as ligands for NR1H2 and NR1H3 nuclear receptors, modulating the transcription of genes involved in lipid metabolism (PubMed:9660774, PubMed:12077124). Plays a role in cholestanol metabolism in the cerebellum. Similarly to cholesterol, hydroxylates cholestanol and may facilitate sterol diffusion through the blood-brain barrier to the systemic circulation for further degradation (PubMed:28190002). Also hydroxylates retinal 7-ketocholesterol, a noxious oxysterol with pro-inflammatory and pro-apoptotic effects, and may play a role in its elimination from the retinal pigment epithelium (PubMed:21411718). May play a redundant role in vitamin D biosynthesis. Catalyzes 25-hydroxylation of vitamin D3 that is required for its conversion to a functionally active form (PubMed:15465040).
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Uniprot ID
Primary ID:
Q02318

Secondarily ID:
A8K303
Q6LDB4
Q86YQ6
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Ensembl ID
ENSG00000135929
HGNC ID
HGNC:2605
Subcellular Location
Mitochondrion inner membrane
Map of Molecular Bioactivity Related to the Protein
Map of Molecular Bioactivity Related to the Protein

Protein
Cell Line
Compound

Bioactivity Value:

<= 0.1 μM
> 0.1 μM and <= 10 μM
> 10 μM
Imprecise Activity
Table of Molecular Bioactivities Related to the Protein
Cell Line ID: CL000011 , CHO
Compound ID Compound Name Compound Formula
CP0469828
2-(2-Bromobenzyl)-6-methoxy-tetralone
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C18H17BrO2
 1
1
IC50 = 59 nM
   TI
   LI
   LO
   TS
CP0427441
2-[1-(2-Bromo-phenyl)-meth-(E)-ylidene]-6-methoxy-3,4-dihydro-2H-naphthalen-1-one
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C18H15BrO2
 1
1
IC50 = 4330 nM
   TI
   LI
   LO
   TS
CP0467416
2-(2-Trifluoromethylbenzyl)-6-methoxy-tetralone
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C19H17F3O2
 1
1
IC50 = 5340 nM
   TI
   LI
   LO
   TS
CP0504379
(E)-2-(2-(E)-Styrylbenzylidene)-6-methoxy-tetralone
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C26H22O2
 1
1
IC50 = 5690 nM
   TI
   LI
   LO
   TS
CP0467417
2-(2-Ethylbenzyl)-6-methoxy-tetralone
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C20H22O2
 1
1
IC50 = 8230 nM
   TI
   LI
   LO
   TS