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)
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
HSA1593(CYP27A1)
PTR459951
PPS100989602(CYP27A1)
GGO101131774
PON100451841
NLE100606147
MCC700192(CYP27A1)
MCF102129822(CYP27A1)
CSAB103217882(CYP27A1)
CATY105587055
PANU100999256
TGE112636454
RRO104660034
RBB108518476
TFN117094510
PTEH111548691
CJC100393783(CYP27A1)
SBQ101029011
CSYR103261235
MMUR105857702
OGA100948265
MMU104086(Cyp27a1)
MCAL110290578
MPAH110322436
RNO301517(Cyp27a1)
MCOC116088582
MUN110566769
CGE100751584
MAUA101842686
PLEU114709306
MORG121447473
NGI103727727
HGL101700018
CPOC100725007
CCAN109679372
DORD105987202
DSP122107505
NCAR124981023
OCU100348736(CYP27A1)
OPI101518668
TUP102471009
CFA610489(CYP27A1)
VVP112922543
VLG121480575
AML100480153
UMR103662754
UAH113250446
UAR123779428
ELK111142401
LLV125095290
MPUF101690391
ORO101374314
EJU114200186
ZCA113919618
MLX118012034
FCA101091962
PYU121031077
PBG122481780
PTG102958835
PPAD109265055
AJU106970103
HHV120232403
BTA511960(CYP27A1)
BOM102275770
BIU109570716
BBUB102408966
CHX102177672
OAS101111528
ODA120854365
CCAD122426554
SSC100126282(CYP27A1)
CFR102517717
CBAI105064023
CDK105096090
VPC102536104
BACU102999025
LVE103080163
OOR101288855
DLE111171838
PCAD102992597
PSIU116756897
ECB100055936(CYP27A1)
EPZ103567914
EAI106838458
MYB102255768
MYD102761397
MMYO118661548
MLF102424757
MNA107524820
PKL118717011
HAI109380589
DRO112307709
SHON118975441
AJM119056899
PDIC114495561
PHAS123829715
MMF118625177
RFQ117025889
PALE102883032
PGIG120587665
PVP105291113
RAY107516735
MJV108390517
TOD119256216
SARA101541473
LAV100662261
TMU101347420
DNM101411078 101411838 101423385
MDO100011045(CYP27A1)
GAS123242736
SHR100918281
PCW110219429
OAA100093207
GGA431683(CYP27A1)
PCOC116227314
MGP100539204
CJO107317020
NMEL110400531
APLA101802865
ACYG106039314
AFUL116490905
TGU100232323
LSR110468382
SCAN103814667
PMOA120496874
OTC121335742
PRUF121356764
GFR102036968
FAB101815213
PHI102102670
PMAJ107207520
CCAE111932181
CCW104693966
ETL114055287
ZAB102062293
FPG101912381
FCH102046779
CLV102085729
EGZ104132432
NNI104010100
ACUN113482393
TALA116959647
PADL103917680
ACHC115342636
AAM106499073
AROW112972033
NPD112959865
DNE112980321
ASN102377709
AMJ102562954
CPOO109322499
GGN109301588
CMY102937044
CPIC101943409
CABI116832589
MRV120374801
ACS100565025
PVT110078231
SUND121935066
PBI103054208
PMUR107292512
TSR106540772
PGUT117675268
VKO123027531
PMUA114603027
ZVI118090854
GJA107116962 107122111
STOW125425853 125427460
XLA100381137(cyp27a1) 108702937 446987(XB984297.L)
XTR100145331 100496109 116407844 594905
NPR108783869 108783870 108783871 108783872 108783878 108783885
RTEM120944205 120944206 120944214
BBUF121007459 121007461 121008616
BGAR122944797 122944805 122945772
DRE322341(cyp27a1.4) 402831(cyp27a7) 558239(si:ch211-113j14.1) 565876(si:dkey-91i10.3) 723999(zgc:136333) 795106(cyp27a1.2)
SRX107705485 107706665 107714212 107723676 107725254 107741910
SANH107658292 107659991 107660625 107660626 107677609 107683415
SGH107555015 107555356 107555927 107578255 107581419 107584802 107596087
CCAR109064887 109072531 109088722 109088733 109088804 109096773 109113433
CAUA113050872 113053045 113053331 113092289 113108126 113108393 113108395 113108503
PPRM120461532 120461533 120466971(si:dkey-91i10.3) 120491553(si:ch211-113j14.1)
MAMB125270463(si:dkey-91i10.3) 125270558 125270559 125270561 125270562 125274236(si:ch211-113j14.1)
IPU108261037 108261972 108266301 108266489 108266490 108266491 108272717(si:ch211-113j14.1)
PHYP113526131 113526132 113526133 113526134 113526210 113526267 113538157
SMEO124380536 124382704 124383131 124383132 124383214(si:dkey-91i10.3) 124392227(si:ch211-113j14.1)
TFD113651034(si:ch211-113j14.1) 113654303 113654508(si:dkey-91i10.3) 113654626
AMEX103023980 103024291 103027002 103036363
EEE113567700 113577222 113586367
TRU101062671 101063777 101069976
TNGGSTEN00029207G001
LCO104934615 104939434 109141591
NCC104944399 104954736 104955625
CGOB115017336 115026484 115026794
ELY117249878(si:ch211-113j14.1) 117251494(si:dkey-91i10.3) 117251800
PLEP121949258(si:dkey-91i10.3) 121949317 121962951(si:ch211-113j14.1)
SLUC116033996(si:dkey-91i10.3) 116044772(si:ch211-113j14.1)
ECRA117939647(si:ch211-113j14.1) 117952801(si:dkey-91i10.3)
PFLV114550996 114564588
GAT120814973 120814975 120834098
PPUG119220421(si:ch211-113j14.1) 119228308 119228466(si:dkey-91i10.3)
MSAM119900367(si:ch211-113j14.1) 119903811 119904427(si:dkey-91i10.3)
CUD121506513(si:ch211-113j14.1) 121522777 121522982(si:dkey-91i10.3)
ALAT119015239(si:ch211-113j14.1) 119025758(si:dkey-91i10.3) 119026140
MZE101465003 101473743 101478374
ONL100691854 100693469 100705229
OAU116314781(si:dkey-91i10.3) 116317150(si:ch211-113j14.1) 116334070
OLA101156051 101160538 101175691
OML112154786 112155582(si:dkey-91i10.3) 112156772(si:ch211-113j14.1)
XMA102225514 102230910 102234678 102234938
XCO114140560 114147791 114147794 114148371
XHE116715672 116722946 116723684 116723685
PRET103456753 103461862 103461863 103478550
PFOR103134814 103138135 103154130
PLAI106945401 106945403 106949526 106956502
PMEI106916312 106919596 106926480
GAF122826962 122840052 122840053 122840415(si:dkey-91i10.3)
CVG107086204 107086205 107095844 107096820
CTUL119774007 119774012 119778495(si:dkey-91i10.3) 119794638(si:ch211-113j14.1)
GMU124861938(si:ch211-113j14.1) 124870981 124871015 124871107(si:dkey-91i10.3)
NFU107384645 107389662 107396451
KMR108238813(si:dkey-91i10.3) 108244283
ALIM106511198 106511262 106514257 106532599
NWH119419001 119419472(si:dkey-91i10.3) 119429051(si:ch211-113j14.1)
AOCE111562629 111568532 111571431 111574457
CSEM103380768 103389542
POV109637350 109640203 109641449
SSEN122765461(si:ch211-113j14.1) 122783812 122784640(si:dkey-91i10.3)
HHIP117755070(si:dkey-91i10.3) 117755238 117778068(si:ch211-113j14.1)
HSP118103627(si:ch211-113j14.1) 118120133 118120203(si:dkey-91i10.3)
LCF108873048 108897118 108897170
SDU111218457 111218691 111222696
SLAL111653799 111667407 111672420
XGL120798148(si:ch211-113j14.1) 120801357(si:dkey-91i10.3) 120801379
HCQ109517561 109519132 109528391
BPEC110165678 110166041 110171370
MALB109955654 109972640 109974026
BSPL114845813(si:ch211-113j14.1) 114846707 114846743(si:dkey-91i10.3)
SASA106574240 106575170 106586130 106586420 106586421
OTW112225125 112235263 112238608(si:dkey-91i10.3) 112253824 112266509
OMY110495869 110501573 110520006 110520277(si:dkey-91i10.3) 110527483
OGO124008274 124010863 124041765 124043781 124043995(si:dkey-91i10.3)
ONE115107908 115110822 115129706 115133470 115136440
SALP111953394 111959818 111970920 111973814
SNH120033627(si:ch211-113j14.1) 120050736 120058201 120058325 120058328 120064454
ELS105016129 105016181 105019951
SFM108928920 108936404
PKI111840728 111851734
AANG118214017(si:ch211-113j14.1) 118222144(si:dkey-91i10.3) 118222523 118223580
LOC102687850 102689682 102690084 102695871
PSPA121322499 121323017 121323418 121323529(si:dkey-91i10.3)
ARUT117404073 117408072 117411922 117426218
LCM102347924 102352926 102355859(EAF2) 102357152
CMK103189020(si:dkey-91i10.3)
RTP109912644 109924020
BBEL109482768
CELCELE_T13C5.1(daf-9)
CBRCBG_10440 CBG_14860(Cbr-daf-9)
BMYBM_BM2557(Bma-daf-9)
LOALOAG_03116
GAE121371591 121371592 121372413
HRJ124282300 124282301
ATEN116287947
 » 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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