25-hydroxyvitamin D-1 alpha hydroxylase, mitochondrial (EC 1.14.15.18) (25-OHD-1 alpha-hydroxylase) (25-hydroxyvitamin D(3) 1-alpha-hydroxylase) (VD3 1A hydroxylase) (Calcidiol 1-monooxygenase) (Cytochrome P450 subfamily XXVIIB polypeptide 1) (Cytochrome P450C1 alpha) (Cytochrome P450VD1-alpha) (Cytochrome p450 27B1)
1_MTQTL 6_ KYASR 11_ VFHRV 16_ RWAPE 21_ LGASL 26_ GYREY 31_ HSARR 36_ SLADI 41_ PGPST 46_ PSFLA 51_ ELFCK 56_ GGLSR 61_ LHELQ 66_ VQGAA 71_ HFGPV 76_ WLASF 81_ GTVRT 86_ VYVAA 91_ PALVE 96_ ELLRQ 101_ EGPRP 106_ ERCSF 111_ SPWTE 116_ HRRCR 121_ QRACG 126_ LLTAE 131_ GEEWQ 136_ RLRSL 141_ LAPLL 146_ LRPQA 151_ AARYA 156_ GTLNN 161_ VVCDL 166_ VRRLR 171_ RQRGR 176_ GTGPP 181_ ALVRD 186_ VAGEF 191_ YKFGL 196_ EGIAA 201_ VLLGS 206_ RLGCL 211_ EAQVP 216_ PDTET 221_ FIRAV 226_ GSVFV 231_ STLLT 236_ MAMPH 241_ WLRHL 246_ VPGPW 251_ GRLCR 256_ DWDQM 261_ FAFAQ 266_ RHVER 271_ REAEA 276_ AMRNG 281_ GQPEK 286_ DLESG 291_ AHLTH 296_ FLFRE 301_ ELPAQ 306_ SILGN 311_ VTELL 316_ LAGVD 321_ TVSNT 326_ LSWAL 331_ YELSR 336_ HPEVQ 341_ TALHS 346_ EITAA 351_ LSPGS 356_ SAYPS 361_ ATVLS 366_ QLPLL 371_ KAVVK 376_ EVLRL 381_ YPVVP 386_ GNSRV 391_ PDKDI 396_ HVGDY 401_ IIPKN 406_ TLVTL 411_ CHYAT 416_ SRDPA 421_ QFPEP 426_ NSFRP 431_ ARWLG 436_ EGPTP 441_ HPFAS 446_ LPFGF 451_ GKRSC 456_ MGRRL 461_ AELEL 466_ QMALA 471_ QILTH 476_ FEVQP 481_ EPGAA 486_ PVRPK 491_ TRTVL 496_ VPERS 501_INLQF
1: A cytochrome P450 monooxygenase involved in vitamin D metabolism and in calcium and phosphorus homeostasis. Catalyzes the rate-limiting step in the activation of vitamin D in the kidney, namely the hydroxylation of 25-hydroxyvitamin D3/calcidiol at the C1alpha-position to form the hormonally active form of vitamin D3, 1alpha,25-dihydroxyvitamin D3/calcitriol that acts via the vitamin D receptor (VDR) (PubMed:10518789, PubMed:10566658, PubMed:12050193, PubMed:22862690, PubMed:9486994). Has 1alpha-hydroxylase activity on vitamin D intermediates of the CYP24A1-mediated inactivation pathway (PubMed:10518789, PubMed:22862690). Converts 24R,25-dihydroxyvitamin D3/secalciferol to 1-alpha,24,25-trihydroxyvitamin D3, an active ligand of VDR. Also active on 25-hydroxyvitamin D2 (PubMed:10518789). Mechanistically, uses molecular oxygen inserting one oxygen atom into a substrate, and reducing the second into a water molecule, with two electrons provided by NADPH via FDXR/adrenodoxin reductase and FDX1/adrenodoxin (PubMed:22862690)