KEGG   ENZYME: 1.14.15.15
Entry
EC 1.14.15.15               Enzyme                                 
Name
cholestanetriol 26-monooxygenase;
5beta-cholestane-3alpha,7alpha,12alpha-triol 26-hydroxylase;
5beta-cholestane-3alpha,7alpha,12alpha-triol hydroxylase;
cholestanetriol 26-hydroxylase;
sterol 27-hydroxylase;
sterol 26-hydroxylase;
cholesterol 27-hydroxylase;
CYP27A;
CYP27A1;
cytochrome P450 27A1'
Class
Oxidoreductases;
Acting on paired donors, with incorporation or reduction of molecular oxygen;
With reduced iron-sulfur protein as one donor, and incorporation of one atom of oxygen into the other donor
Sysname
5beta-cholestane-3alpha,7alpha,12alpha-triol,adrenodoxin:oxygen oxidoreductase (26-hydroxylating)
Reaction(IUBMB)
5beta-cholestane-3alpha,7alpha,12alpha-triol + 6 reduced adrenodoxin + 6 H+ + 3 O2 = (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate + 6 oxidized adrenodoxin + 4 H2O (overall reaction) [RN:R11142];
(1a) 5beta-cholestane-3alpha,7alpha,12alpha-triol + 2 reduced adrenodoxin + 2 H+ + O2 = (25R)-5beta-cholestane-3alpha,7alpha,12alpha,26-tetraol + 2 oxidized adrenodoxin + H2O [RN:R04807];
(1b) (25R)-5beta-cholestane-3alpha,7alpha,12alpha,26-tetraol + 2 reduced adrenodoxin + 2 H+ + O2 = (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-al + 2 oxidized adrenodoxin + 2 H2O [RN:R03507];
(1c) (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-al + 2 reduced adrenodoxin + 2 H+ + O2 = (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate + 2 oxidized adrenodoxin + H2O [RN:R08761]
Reaction(KEGG)
R03507 R04807 R08761 R11142;
(other) R04806 R08505 R08758 R08759 R08760
Substrate
5beta-cholestane-3alpha,7alpha,12alpha-triol [CPD:C05454];
reduced adrenodoxin [CPD:C00662];
H+ [CPD:C00080];
O2 [CPD:C00007];
(25R)-5beta-cholestane-3alpha,7alpha,12alpha,26-tetraol;
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-al
Product
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate;
oxidized adrenodoxin [CPD:C00667];
H2O [CPD:C00001];
(25R)-5beta-cholestane-3alpha,7alpha,12alpha,26-tetraol;
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-al
Comment
This mitochondrial cytochrome P-450 enzyme requires adrenodoxin. It catalyses the first three sterol side chain oxidations in bile acid biosynthesis via the neutral (classic) pathway. Can also act on cholesterol, cholest-5-ene-3beta,7alpha-diol, 7alpha-hydroxycholest-4-en-3-one, and 5beta-cholestane-3alpha,7alpha-diol. The enzyme can also hydroxylate cholesterol at positions 24 and 25. The initial source of the electrons is NADPH, which transfers the electrons to the adrenodoxin via EC 1.18.1.6, adrenodoxin-NADP+ reductase.
History
EC 1.14.15.15 created 1976 as EC 1.14.13.15, modified 2005, modified 2012, transferred 2016 to EC 1.14.15.15
Pathway
ec00120  Primary bile acid biosynthesis
ec01100  Metabolic pathways
Orthology
K00488  cholestanetriol 26-monooxygenase
K22878  cytochrome P450 daf-9
Genes
HSA: 1593(CYP27A1)
PTR: 459951
PPS: 100989602(CYP27A1)
GGO: 101131774
PON: 100451841
NLE: 100606147
HMH: 116810284
MCC: 700192(CYP27A1)
MCF: 102129822(CYP27A1)
MTHB: 126932993
MNI: 105481230
CSAB: 103217882(CYP27A1)
CATY: 105587055
PANU: 100999256
TGE: 112636454
MLEU: 105528099
RRO: 104660034
RBB: 108518476
TFN: 117094510
PTEH: 111548691
CANG: 105504716
CJC: 100393783(CYP27A1)
SBQ: 101029011
CIMI: 108309072(CYP27A1)
CSYR: 103261235
MMUR: 105857702
LCAT: 123643999
PCOQ: 105808833
OGA: 100948265
MMU: 104086(Cyp27a1)
MCAL: 110290578
MPAH: 110322436
RNO: 301517(Cyp27a1)
MCOC: 116088582
ANU: 117705443
MUN: 110566769
CGE: 100751584
MAUA: 101842686
PROB: 127226444
PLEU: 114709306
MORG: 121447473
MFOT: 126492940
AAMP: 119820452
NGI: 103727727
HGL: 101700018
CPOC: 100725007
CCAN: 109679372
DORD: 105987202
DSP: 122107505
PLOP: 125350293
NCAR: 124981023
MMMA: 107138401
OCU: 100348736(CYP27A1)
OPI: 101518668
TUP: 102471009
GVR: 103600243
CFA: 610489(CYP27A1)
CLUD: 112656756
VVP: 112922543
VLG: 121480575
NPO: 129523037
AML: 100480153
UMR: 103662754
UAH: 113250446
UAR: 123779428
ELK: 111142401
LLV: 125095290
MPUF: 101690391
MNP: 132013556
MLK: 131827446
NVS: 122902315
ORO: 101374314
EJU: 114200186
ZCA: 113919618
MLX: 118012034
NSU: 110592102
LWW: 102750264
FCA: 101091962(CYP27A1)
PYU: 121031077
PCOO: 112861202
PBG: 122481780
PVIV: 125173721
LRUF: 124516601
PTG: 102958835
PPAD: 109265055
PUC: 125911174
AJU: 106970103
HHV: 120232403
BTA: 511960(CYP27A1)
BOM: 102275770
BIU: 109570716
BBUB: 102408966
BBIS: 105003777
CHX: 102177672
OAS: 101111528
BTAX: 128043348
ODA: 120854365
CCAD: 122426554
MBEZ: 129537809
SSC: 100126282(CYP27A1)
CFR: 102517717
CBAI: 105064023
CDK: 105096090
VPC: 102536104
BACU: 102999025
BMUS: 118897921
LVE: 103080163
OOR: 101288855
DLE: 111171838
PCAD: 102992597
PSIU: 116756897
NASI: 112411895
ECB: 100055936(CYP27A1)
EPZ: 103567914
EAI: 106838458
MYB: 102255768
MYD: 102761397
MMYO: 118661548
MLF: 102424757
PKL: 118717011
EFUS: 103289402
MNA: 107524820
DRO: 112307709
SHON: 118975441
AJM: 119056899
PDIC: 114495561
PHAS: 123829715
MMF: 118625177
PPAM: 129080340
HAI: 109380589
RFQ: 117025889
PALE: 102883032
PGIG: 120587665
PVP: 105291113
RAY: 107516735
MJV: 108390517
TOD: 119256216
SARA: 101541473
SETR: 126000867
LAV: 100662261
TMU: 101347420
ETF: 101661290
DNM: 101411078 101411838 101423385
MDO: 100011045(CYP27A1)
GAS: 123242736
SHR: 100918281
AFZ: 127553095
PCW: 110219429
OAA: 100093207
GGA: 431683(CYP27A1)
PCOC: 116227314
MGP: 100539204
CJO: 107317020
TPAI: 128077280
LMUT: 125697173
NMEL: 110400531
APLA: 101802865
ACYG: 106039314
CATA: 118252615
AFUL: 116490905
TGU: 100232323
LSR: 110468382
SCAN: 103814667
PMOA: 120496874
OTC: 121335742
PRUF: 121356764
GFR: 102036968
FAB: 101815213
OMA: 130255402
PHI: 102102670
PMAJ: 107207520
CCAE: 111932181
CCW: 104693966
CBRC: 103611331
ETL: 114055287
ZAB: 102062293
ACHL: 103804610
SVG: 106860300
MMEA: 130574379
HRT: 120754967
FPG: 101912381
FCH: 102046779
CLV: 102085729
EGZ: 104132432
NNI: 104010100
PCRI: 104034046
PLET: 104625871
EHS: 104505705
ACUN: 113482393
TALA: 116959647
PADL: 103917680
AFOR: 103895441
ACHC: 115342636
HLE: 104833136
AGEN: 126044548
GCL: 127018337
CCRI: 104164669
CSTI: 104560122
CMAC: 104485562
MUI: 104542244
BREG: 104639071
FGA: 104076041
LDI: 104354355
MNB: 103771183
OHA: 104339178
NNT: 104404639
SHAB: 115608939
ACAR: 104522074
CPEA: 104387486
AVIT: 104271934
CVF: 104285520
RTD: 128913064
CUCA: 104056625
TEO: 104381960
AAM: 106499073
AROW: 112972033
NPD: 112959865
TGT: 104568471
DNE: 112980321
SCAM: 104153734
ASN: 102377709
AMJ: 102562954
CPOO: 109322499
GGN: 109301588
CMY: 102937044
CCAY: 125644577
DCC: 119863469
CPIC: 101943409
CABI: 116832589
MRV: 120374801
ACS: 100565025
ASAO: 132773757
PVT: 110078231
SUND: 121935066
PBI: 103054208
PMUR: 107292512
CTIG: 120318333
TSR: 106540772
PGUT: 117675268
PTEX: 113441122
NSS: 113410820
VKO: 123027531
PMUA: 114603027
PRAF: 128420161
ZVI: 118090854
HCG: 128340098 128340957
GJA: 107116962 107122111
STOW: 125425853 125427460
EMC: 129323825 129323969
XLA: 100381137(cyp27a1) 108702937 446987(XB984297.L)
XTR: 100145331 100496109 116407844 594905
NPR: 108783869 108783870 108783871 108783872 108783878 108783885
RTEM: 120944205 120944206 120944214
BBUF: 121007459 121007461 121008616
BGAR: 122944797 122944805 122945772
MUO: 115474647
DRE: 322341(cyp27a1.4) 402831(cyp27a7) 558239(si:ch211-113j14.1) 565876(cyp27a3) 723999(zgc:136333) 795106(cyp27a1.2)
SRX: 107705485 107706665 107714212 107723676 107725254 107741910
SANH: 107658292 107659991 107660625 107660626 107677609 107683415
SGH: 107555015 107555356 107555927 107578255 107581419 107584802 107596087
CCAR: 109064887 109072531 109088722 109088733 109088804 109096773 109113433
CAUA: 113050872 113053045 113053331 113092289 113108126 113108393 113108395 113108503
CGIB: 127959978(si:ch211-113j14.1) 127964813 127965339 128016157 128019738 128020024
PTET: 122346890(si:ch211-113j14.1) 122351411(si:dkey-91i10.3) 122351848 122351850
LROH: 127166631 127171000 127171002 127171405
OMC: 131542011 131546658 131547049 131547580
PPRM: 120461532 120461533 120466971(si:dkey-91i10.3) 120491553(si:ch211-113j14.1)
RKG: 130085900 130096620 130096621 130101313
MAMB: 125270463(si:dkey-91i10.3) 125270558 125270559 125270561 125270562 125274236(si:ch211-113j14.1)
CIDE: 127514128(si:ch211-113j14.1) 127518365 127518366 127518368(si:dkey-91i10.3) 127518425
MASI: 127446272(si:ch211-113j14.1) 127447105(si:dkey-91i10.3) 127447172 127448309 127448847
TROS: 130555464 130556084 130556649
TDW: 130424684 130427058 130427607
MANU: 129451170 129451484 129452197 129452198
IPU: 108261037 108261972 108266489 108266490 108266491 108272717(si:ch211-113j14.1) 124627672
IFU: 128608666 128609022 128609023 128609024 128615288
PHYP: 113526131 113526132 113526133 113526134 113526210 113526267 113538157
SMEO: 124380536 124382704 124383131 124383132 124383214(si:dkey-91i10.3) 124392227(si:ch211-113j14.1)
TFD: 113651034(si:ch211-113j14.1) 113654303 113654508(si:dkey-91i10.3) 113654626
TVC: 132844928 132850104 132850336 132850337
AMEX: 103023980 103024291 103027002 103036363
CMAO: 118807247 118807846(si:ch211-113j14.1) 118824141(si:dkey-91i10.3)
EEE: 113567700 113577222 113586367
CHAR: 105892637(si:ch211-113j14.1) 105907897 105912667(si:dkey-91i10.3)
TRU: 101062671 101063777 101069976
TFS: 130522346 130530915 130534294
TNG: GSTEN00029207G001
LCO: 104934615 104939434 109141591
NCC: 104944399 104954736 104955625
TBEN: 117485949 117495315(si:dkey-91i10.3) 117499195(si:ch211-113j14.1)
CGOB: 115017336 115026484 115026794
PGEO: 117441076 117466440(si:dkey-91i10.3) 117466930
GACU: 117533149(si:ch211-113j14.1) 117538127(si:dkey-91i10.3) 117549511
EMAC: 134860628 134867093 134867838
ELY: 117249878(si:ch211-113j14.1) 117251494(si:dkey-91i10.3) 117251800
EFO: 125884826(si:ch211-113j14.1) 125899310(si:dkey-91i10.3) 125899964
PLEP: 121949258(si:dkey-91i10.3) 121949317 121962951(si:ch211-113j14.1)
SLUC: 116033996(si:dkey-91i10.3) 116044772(si:ch211-113j14.1)
ECRA: 117939647(si:ch211-113j14.1) 117952801(si:dkey-91i10.3)
ESP: 116673863 116697553
PFLV: 114550996 114564588
GAT: 120814973 120814975 120834098
PPUG: 119220421(si:ch211-113j14.1) 119228308 119228466(si:dkey-91i10.3)
AFB: 129104391 129104777 129111512
CLUM: 117741891(si:ch211-113j14.1) 117750380 117750467(si:dkey-91i10.3)
PSWI: 130204495 130207217 130208830
MSAM: 119900367(si:ch211-113j14.1) 119903811 119904427(si:dkey-91i10.3)
SCHU: 122871830(si:ch211-113j14.1) 122885091(si:dkey-91i10.3) 122886090
CUD: 121506513(si:ch211-113j14.1) 121522777 121522982(si:dkey-91i10.3)
ALAT: 119015239(si:ch211-113j14.1) 119025758(si:dkey-91i10.3) 119026140
MZE: 101465003 101473743 101478374
ONL: 100691854 100693469 100705229
OAU: 116314781(si:dkey-91i10.3) 116317150(si:ch211-113j14.1) 116334070
OLA: 101156051 101160538 101175691
OML: 112154786 112155582(si:dkey-91i10.3) 112156772(si:ch211-113j14.1)
CSAI: 133425196 133445710 133446096
XMA: 102225514 102230910 102234678 102234938
XCO: 114140560 114147791 114147794 114148371
XHE: 116715672 116722946 116723684 116723685
PRET: 103456753 103461862 103461863 103478550
PFOR: 103134814 103138135 103154130
PLAI: 106945401 106945403 106949526 106956502
PMEI: 106916312 106919596 106926480
GAF: 122826962 122840052 122840053 122840415(si:dkey-91i10.3)
PPRL: 129355976 129360843 129374479 129374480
CVG: 107086204 107086205 107095844 107096820
CTUL: 119774007 119774012 119778495(si:dkey-91i10.3) 119794638(si:ch211-113j14.1)
GMU: 124861938(si:ch211-113j14.1) 124870981 124871015 124871107(si:dkey-91i10.3)
NFU: 107384645 107389662 107396451
KMR: 108238813(si:dkey-91i10.3) 108244283
ALIM: 106511198 106511262 106514257 106532599
NWH: 119419001 119419472(si:dkey-91i10.3) 119429051(si:ch211-113j14.1)
AOCE: 111562629 111568532 111571431 111574457
MCEP: 125000998 125018222(si:dkey-91i10.3) 125018282
CSEM: 103380768 103389542
POV: 109637350 109640203 109641449
SSEN: 122765461(si:ch211-113j14.1) 122783812 122784640(si:dkey-91i10.3)
HHIP: 117755070(si:dkey-91i10.3) 117755238 117778068(si:ch211-113j14.1)
HSP: 118103627(si:ch211-113j14.1) 118120133 118120203(si:dkey-91i10.3)
SMAU: 118282520(si:dkey-91i10.3) 118283128 118299537(si:ch211-113j14.1)
LCF: 108873048(si:ch211-113j14.1) 108897118(si:dkey-91i10.3) 108897170
SDU: 111218457 111218691 111222696
SLAL: 111653799 111667407 111672420
XGL: 120798148(si:ch211-113j14.1) 120801357(si:dkey-91i10.3) 120801379
HCQ: 109517561 109519132 109528391
SSCV: 125973467 125973998(si:dkey-91i10.3) 125988453(si:ch211-113j14.1)
SBIA: 133507117 133511790 133512247
PEE: 133403915 133410410 133410778
PTAO: 133480720 133486533 133486856
BPEC: 110165678 110166041 110171370
MALB: 109955654 109972640 109974026
BSPL: 114845813(si:ch211-113j14.1) 114846707 114846743(si:dkey-91i10.3)
SJO: 128354620 128367942 128368386
SASA: 106574240 106575170 106586130 106586420 106586421
STRU: 115156161 115160652 115160949 115160950 115179454 115196033
OTW: 112225125 112235263 112238608(si:dkey-91i10.3) 112253824 112266509
OMY: 110495869 110501573 110520006 110520277(si:dkey-91i10.3) 110527483
OGO: 124008274 124010863 124041765 124043781 124043995(si:dkey-91i10.3)
ONE: 115107908 115110822 115129706 115133470 115136440
OKI: 109865639 109868100 109870843 109878202 109906858
OKE: 118363755 118366908 118367168 118370220 118385931
SALP: 111953394 111959818 111970920 111973814
SNH: 120033627(si:ch211-113j14.1) 120050736 120058201 120058325 120058328 120064454
CCLU: 121536654 121537015 121548322 123481257
ELS: 105016129 105016181 105019951
SFM: 108928920 108936404
PKI: 111840728 111851734
AANG: 118214017(si:ch211-113j14.1) 118222144(si:dkey-91i10.3) 118222523 118223580
LOC: 102687850 102689682 102690084 102695871
PSPA: 121322499 121323017 121323418 121323529(si:dkey-91i10.3)
ARUT: 117404073 117408072 117411922 117426218
PSEX: 120530726(si:dkey-91i10.3) 120531223 120531279(si:ch211-113j14.1)
LCM: 102347924 102352926 102355859(EAF2) 102357152
CMK: 103189020(si:dkey-91i10.3)
RTP: 109912644(si:dkey-91i10.3) 109924020
CPLA: 122551787(si:dkey-91i10.3) 122551956
HOC: 132817700 132817724
LERI: 129693908
PMRN: 116938626 116940874
LRJ: 133344490 133344639 133346016
BBEL: 109482768
LPIC: 129255121
CEL: CELE_T13C5.1(daf-9)
CBR: CBG_10440 CBG_14860(Cbr-daf-9)
CRQ: GCK72_024534
BMY: BM_BM2557(Bma-daf-9)
LOA: LOAG_03116
GAE: 121371591 121371592 121372413
HRJ: 124282300 124282301
OED: 125650244
ATEN: 116287947
 » show all
Reference
1  [PMID:5914340]
  Authors
Masui T, Herman R, Staple E.
  Title
The oxidation of 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha, 26-tetraol to 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol-26-oic acid via 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol-26-al by rat liver.
  Journal
Biochim Biophys Acta 117:266-8 (1966)
DOI:10.1016/0304-4165(66)90177-2
Reference
2  [PMID:4388637]
  Authors
Okuda K, Hoshita N.
  Title
Oxidation of 5-beta-cholestane-3 alpha, 7 alpha, 12 alpha-triol by rat-liver mitochondria.
  Journal
Biochim Biophys Acta 164:381-8 (1968)
DOI:10.1016/0005-2760(68)90162-8
Reference
3  [PMID:6423637]
  Authors
Wikvall K.
  Title
Hydroxylations in biosynthesis of bile acids. Isolation of a cytochrome P-450 from rabbit liver mitochondria catalyzing 26-hydroxylation of C27-steroids.
  Journal
J Biol Chem 259:3800-4 (1984)
Reference
4  [PMID:2722778]
  Authors
Andersson S, Davis DL, Dahlback H, Jornvall H, Russell DW
  Title
Cloning, structure, and expression of the mitochondrial cytochrome P-450 sterol 26-hydroxylase, a bile acid biosynthetic enzyme.
  Journal
J Biol Chem 264:8222-9 (1989)
  Sequence
[ocu:100348736]
Reference
5  [PMID:2322231]
  Authors
Dahlback H, Holmberg I
  Title
Oxidation of 5 beta-cholestane-3 alpha,7 alpha, 12 alpha-triol into 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid by cytochrome P-450(26) from rabbit liver mitochondria.
  Journal
Biochem Biophys Res Commun 167:391-5 (1990)
DOI:10.1016/0006-291X(90)92034-W
Reference
6  [PMID:8496170]
  Authors
Holmberg-Betsholtz I, Lund E, Bjorkhem I, Wikvall K.
  Title
Sterol 27-hydroxylase in bile acid biosynthesis. Mechanism of oxidation of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,27-tetrol into 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid.
  Journal
J Biol Chem 268:11079-85 (1993)
Reference
7  [PMID:9660774]
  Authors
Pikuleva IA, Babiker A, Waterman MR, Bjorkhem I
  Title
Activities of recombinant human cytochrome P450c27 (CYP27) which produce intermediates of alternative bile acid biosynthetic pathways.
  Journal
J Biol Chem 273:18153-60 (1998)
DOI:10.1074/jbc.273.29.18153
Reference
8  [PMID:10512735]
  Authors
Furster C, Bergman T, Wikvall K.
  Title
Biochemical characterization of a truncated form of CYP27A purified from rabbit liver mitochondria.
  Journal
Biochem Biophys Res Commun 263:663-6 (1999)
DOI:10.1006/bbrc.1999.1426
Reference
9  [PMID:11412116]
  Authors
Pikuleva IA, Puchkaev A, Bjorkhem I
  Title
Putative helix F contributes to regioselectivity of hydroxylation in mitochondrial cytochrome P450 27A1.
  Journal
Biochemistry 40:7621-9 (2001)
DOI:10.1021/bi010193i
  Sequence
[hsa:1593]
Other DBs
ExplorEnz - The Enzyme Database: 1.14.15.15
IUBMB Enzyme Nomenclature: 1.14.15.15
ExPASy - ENZYME nomenclature database: 1.14.15.15
BRENDA, the Enzyme Database: 1.14.15.15
CAS: 52227-77-7

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