Sterol 26-hydroxylase, mitochondrial (EC 1.14.15.15) (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)
1_MAALG 6_ CARLR 11_ WALRG 16_ AGRGL 21_ CPHGA 26_ RAKAA 31_ IPAAL 36_ PSDKA 41_ TGAPG 46_ AGPGV 51_ RRRQR 56_ SLEEI 61_ PRLGQ 66_ LRFFF 71_ QLFVQ 76_ GYALQ 81_ LHQLQ 86_ VLYKA 91_ KYGPM 96_ WMSYL 101_ GPQMH 106_ VNLAS 111_ APLLE 116_ QVMRQ 121_ EGKYP 126_ VRNDM 131_ ELWKE 136_ HRDQH 141_ DLTYG 146_ PFTTE 151_ GHHWY 156_ QLRQA 161_ LNQRL 166_ LKPAE 171_ AALYT 176_ DAFNE 181_ VIDDF 186_ MTRLD 191_ QLRAE 196_ SASGN 201_ QVSDM 206_ AQLFY 211_ YFALE 216_ AICYI 221_ LFEKR 226_ IGCLQ 231_ RSIPE 236_ DTVTF 241_ VRSIG 246_ LMFQN 251_ SLYAT 256_ FLPKW 261_ TRPVL 266_ PFWKR 271_ YLDGW 276_ NAIFS 281_ FGKKL 286_ IDEKL 291_ EDMEA 296_ QLQAA 301_ GPDGI 306_ QVSGY 311_ LHFLL 316_ ASGQL 321_ SPREA 326_ MGSLP 331_ ELLMA 336_ GVDTT 341_ SNTLT 346_ WALYH 351_ LSKDP 356_ EIQEA 361_ LHEEV 366_ VGVVP 371_ AGQVP 376_ QHKDF 381_ AHMPL 386_ LKAVL 391_ KETLR 396_ LYPVV 401_ PTNSR 406_ IIEKE 411_ IEVDG 416_ FLFPK 421_ NTQFV 426_ FCHYV 431_ VSRDP 436_ TAFSE 441_ PESFQ 446_ PHRWL 451_ RNSQP 456_ ATPRI 461_ QHPFG 466_ SVPFG 471_ YGVRA 476_ CLGRR 481_ IAELE 486_ MQLLL 491_ ARLIQ 496_ KYKVV 501_ LAPET 506_ GELKS 511_ VARIV 516_ LVPNK 521_ KVGLQ 526_FLQRQ
1: 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:12077124, PubMed:21411718, PubMed:28190002, PubMed:9660774). Regulates cholesterol homeostasis by catalyzing the conversion of excess cholesterol to bile acids via both the 'neutral' (classic) and the 'acid' (alternative) pathways (PubMed:11412116, PubMed:1708392, PubMed:2019602, PubMed:7915755, PubMed:9186905, PubMed:9660774, 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:12077124, PubMed:9660774). 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)