Sterol 26-Hydroxylase,Mitochondrial

E3869Hu

Sandwich

48T,96T

4.89ng/L

10-4000ng/L

Serum, plasma, cell culture supernates

Human

2-8ºC

1h 30m

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).

60,235

1593

5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 26-hydroxylase;CYP27A1;Cytochrome P450 27;Cytochrome P-450C27/25;Sterol 26-hydroxylase, mitochondrial;Sterol 27-hydroxylase;Vitamin D(3 25-hydroxylase

CYP27A1

Cardiovascular

CYP27A1