KEGG ENZYME: 1.14.14.139

ENZYME: 1.14.14.139

Entry

EC 1.14.14.139              Enzyme

Name

5beta-cholestane-3alpha,7alpha-diol 12alpha-hydroxylase;
5beta-cholestane-3alpha,7alpha-diol 12alpha-monooxygenase;
sterol 12alpha-hydroxylase (ambiguous);
CYP8B1;
cytochrome P450 8B1;
7alpha-hydroxycholest-4-en-3-one 12alpha-hydroxylase;
7alpha-hydroxy-4-cholesten-3-one 12alpha-monooxygenase;
chenodeoxycholate 12alpha monooxygenase

Class

Oxidoreductases;
Acting on paired donors, with incorporation or reduction of molecular oxygen;
With reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen into the other donor

Sysname

5beta-cholestane-3alpha,7alpha-diol,[reduced NADPH---hemoprotein reductase]:oxygen oxidoreductase (12alpha-hydroxylating)

Reaction(IUBMB)

(1) 5beta-cholestane-3alpha,7alpha-diol + [reduced NADPH---hemoprotein reductase] + O2 = 5beta-cholestane-3alpha,7alpha,12alpha-triol + [oxidized NADPH---hemoprotein reductase] + H2O [RN:R07204];
(2) 7alpha-hydroxycholest-4-en-3-one + [reduced NADPH---hemoprotein reductase] + O2 = 7alpha,12alpha-dihydroxycholest-4-en-3-one + [oxidized NADPH---hemoprotein reductase] + H2O [RN:R04826];
(3) chenodeoxycholate + [reduced NADPH---hemoprotein reductase] + O2 = cholate + [oxidized NADPH---hemoprotein reductase] + H2O [RN:R12550]

Reaction(KEGG)

R04826 R07204 R12550

Substrate

5beta-cholestane-3alpha,7alpha-diol [CPD:C05452];
[reduced NADPH---hemoprotein reductase] [CPD:C03024];
O2 [CPD:C00007];
7alpha-hydroxycholest-4-en-3-one [CPD:C05455];
chenodeoxycholate [CPD:C02528]

Product

5beta-cholestane-3alpha,7alpha,12alpha-triol [CPD:C05454];
[oxidized NADPH---hemoprotein reductase] [CPD:C03161];
H2O [CPD:C00001];
7alpha,12alpha-dihydroxycholest-4-en-3-one;
cholate [CPD:C00695]

Comment

A cytochrome P-450 (heme-thiolate) protein found in mammals. This is the key enzyme in the biosynthesis of the bile acid cholate. The enzyme can also hydroxylate 5beta-cholestane-3alpha,7alpha-diol at the 25 and 26 position, but to a lesser extent [2].

History

EC 1.14.14.139 created 2005 as EC 1.14.13.96, transferred 2018 to EC 1.14.14.139 (EC 1.14.18.8 created 2005 as EC 1.14.13.95, transferred 2015 to EC 1.14.18.8, incorporated 2020) , modified 2020

Pathway

ec00120

Primary bile acid biosynthesis

ec01100

Metabolic pathways

Orthology

K07431

sterol 12-alpha-hydroxylase

Genes

HSA:	1582(CYP8B1)

PTR:	736962(CYP8B1)

PPS:	100969929(CYP8B1)

GGO:

101147556

PON:

100433242

PPYG:

129033134

NLE:

100605497

HMH:

116473602

SSYN:

129483764

MCC:

717247

MCF:	102132602(CYP8B1)

MTHB:

126948519

MNI:

105480287

CSAB:

103227471(CYP8B1)

CATY:

105573840

PANU:

101002418

TGE:

112618174

MLEU:

105542637

RRO:

104670956

RBB:

108532333

TFN:

117083966

PTEH:

111555603

CANG:

105518110

CJC:	100402181(CYP8B1)

SBQ:

101037790

CIMI:

108300032(CYP8B1)

ANAN:

105729791

CSYR:

103271067

MMUR:

105874433 105874434

PCOQ:

105806025

OGA:

100943894

MMU:	13124(Cyp8b1)

MCAL:

110302470

MPAH:

110327161

RNO:	81924(Cyp8b1)

MCOC:

116073386

ANU:

117693741

ASYL:

127688364

MUN:

110554660

CGE:

100770995

MAUA:

101841117

PROB:

127213322

PLEU:

114707690

MORG:

121464432

MFOT:

126501221

AAMP:

119810738

NGI:

103747146

CPOC:

100734566 100734845

CCAN:

109691739

DORD:

105996287

DSP:

122100712

PLOP:

125342505

NCAR:

124969104

MMMA:

107145454

ITI:

101977223

OCU:	100009301(CYP8B1)

OPI:

101531078

TUP:

102479742

GVR:

103588225

CFA:	485619(CYP8B1)

CLUD:

112668685

VVP:

112921604

VLG:

121478995

NPO:

129507154

AML:

100464768

UMR:

103682072

UAH:

113253657

UAR:

123778630

ELK:

111158988

LLV:

125105604

MPUF:

101678059

MNP:

132011721

MLK:

131824441

NVS:

122910564

ORO:

101374077

EJU:

114212204

ZCA:

113917882

MLX:

117999729

NSU:

110570803

LWW:

102751180

FCA:	101082004(CYP8B1)

PYU:

121033533

PCOO:

112850288

PBG:

122492097

PVIV:

125174942

LRUF:

124526704

LGF:

123575930

AJU:

106966675

PTG:

102956513

PPAD:

109248827

PUC:

125922037

PLEZ:

122230096

HHV:	120243596 120243597

BTA:	538964(CYP8B1)

BOM:	102278686(CYP8B1)

BIU:

109576421

BBUB:

102407955

BBIS:

104985319

CHX:

102173339

OAS:

101119853

BTAX:

128048202 128055603

CSUM:

138087559(CYP8B1)

ODA:

120867731

CCAD:

122425003

MREE:

136167753(CYP8B1)

OVR:	110124068(CYP8B1)

MBEZ:

129548099

SSC:	403328(CYP8B1)

CFR:

102524173

CBAI:

105061779

CDK:

105084606

VPC:

102527710

BACU:

103000983

BMUS:

118904139

LVE:

103085360

OOR:

101285967

LALB:

132528050

DLE:

111164311

PCAD:

102990799

PSIU:

116762132

PPHC:

136129572(CYP8B1)

NASI:

112407426

ECB:

100054970

EPZ:	103559259(CYP8B1)

EAI:

123275565

MYB:

102245777

MYD:

102765424

MMYO:

118668388

MLF:

102440551

MDT:

132215759

MYUM:

138992629(CYP8B1)

PKL:

118715531

EFUS:

103300174

MNA:

107524319

DRO:

112309563

SHON:

118976225

AJM:

119051535

PDIC:

114501955

PHAS:

123830673

MMF:

118632899

PPAM:

129087561

HAI:

109377081

RFQ:

117036539

PALE:

102877747

PGIG:

120581963

PVP:

105290391

RAY:

107519986

MJV:

108397210

TOD:

119258772

SARA:

101553886

SFUM:

130037483

SETR:

126020323

LAV:

100665029

TMU:

101356001

ETF:

101641635

DNM:

101440886

MDO:	100026525 100026549(CYP8B)

GAS:	123230807 123254771

SHR:	100918099 100919664 100926550 105749568 116423310

PCW:

110217059

TVP:	118830858 118831777

PBRV:

138166953 138166954

OAA:

103166427

TACU:

119936267

GGA:	425055(CYP8B1)

PCOC:

116238313

MGP:

100546803

CJO:

107308837

TPAI:

128086424

LMUT:

125696205

NMEL:

110395135

APLA:

101797490

ACYG:

106036789(CYP8B1)

CATA:

118251836

AFUL:

116486814

TGU:

100217899

LSR:	110474519(CYP8B1)

SCAN:

103812684

PMOA:

120510523

OTC:

121338124

PRUF:

121365741

ATRC:

139591337(CYP8B1)

GFR:

102041987

FAB:

101809893

OMA:

130249640

PHI:

102112501

PMAJ:

107201006

CCAE:

111925558

CCW:

104686389

CBRC:

103623922

ACOE:

138105575(CYP8B1)

ETL:

114064499

ZAB:

102072546

ZLE:

135443528

ACHL:

103806276

SVG:

106851620

MMEA:

130576304

HRT:

120761181

SATI:

136358021(CYP8B1)

CCIC:

134044170

FPG:

101924073

FCH:

102052323

CCRI:

104165126

NNT:

104401579

SHAB:

115608225

ACUN:

113476903

TALA:

104358666

ACHC:

115338885

HALD:

104325797

HLE:

104828057

AGEN:

126037987

HHAR:

128151212

GCL:

127013785

CSTI:

104557140

LDI:

104352636

MNB:

103770557

DPUB:

104298110

PPUS:

135185795

AVIT:

104270263

BRHI:

104500507

EGZ:

104124585

NNI:

104021734

PCRI:

104033050

PCAO:

104052603

PADL:

103924714

AFOR:

103896167

FGA:

104080441

GSTE:

104259738

CBOY:

140647198(CYP8B1)

CLV:

102083443

CNIC:

135985854

MUI:

104544950

PGUU:

104472378

PLET:

104619451

EHS:

104515298

CMAC:

104477299

CUCA:

104056957

TEO:

104376951

BREG:

104634046

OHA:

104326246

ACAR:

104530672

CPEA:

104386005

CVF:

104282407

RTD:

128906301

CRID:

134510552

NPD:

112953164

TGT:

104567901

DNE:

112980758

SCAM:

104150126

ASN:

102374897

AMJ:	102573091(CYP8B1)

CPOO:

109306069

GGN:

109291179

PSS:

102444673

CMY:

102934823

CCAY:

125630813

DCC:	119850697 119851812

CPIC:

101950173

TST:

117871942

CABI:

116819555

MRV:

120399358

ACS:	100551676 100551876 100562756 100562953 100565509 100565705 100565905 100566106

ASAO:

132779804 132779808 132779809 132779810

PVT:	110091568 110091574 110091575

SUND:

121932425 121934688 121935162 121935580 121935581

PBI:	103051222 103065731

PMUR:

107284342 107284343 107284344 107284346 107284348 107284362

CTIG:

120296691 120318053 120318058 120318065 120319285

TSR:	106538485 106538492

PGUT:

117680087 117680089

APRI:

131196734 131196735

PTEX:

113449276 113449277

NSS:	113422182 113422190

VKO:	123020905 123020984

PMUA:

114607583 114607705 114607796 114607915

PRAF:

128398296 128398297 128398298

ZVI:	118091758 118091759 118091944

HCG:	128330313 128330314 128330315 128331371 128331373 128331374 128331751 128331752

GJA:	107106985 107106986 107110695 107112093 107125164

STOW:

125440698 125441151

EMC:	129337237 129337817 129337819 129337820 129337853 129337869 129337928

XLA:	108718630(cyp8b1.2.L) 379482(cyp8b1.1.S)

XTR:	100488015(cyp8b1.2) 100488168(cyp8b1.1)

NPR:

108788787

RTEM:

120939823

ACAT:

141144094 141144096

BBUF:

121000933

BGAR:

122929107 122929108

DTC:	141054866 141054868

PWL:	138261719 138261722

MUO:	115466283 115466290 115466320 115466327 115466341 115466365

GSH:

117355598

DRE:	100004274(cyp8b1.2) 445281(cyp8b1.1) 447848(cyp8b1.3)

SRX:	107711691 107727542

SANH:

107661909 107671665

SGH:	107558014 107580338 107593879

CCAR:

109056773 109056779

CAUA:

113038725 113038733 113046101

CGIB:

127950969 128024980 128024994

PTET:

122324044

LROH:

127152816

GRF:

141344059

OMC:

131525180

PPRM:

120477644 120479271

RKG:

130098852

MAMB:

125254255

CIDE:

127504066

CERY:

137002930

MASI:

127440947 127441950

TROS:

130554573

TDW:

130417464

MANU:

129442789 129442794 129442795

PDAB:

135749279 135749284 135749288

IPU:	108261592 108268189 108268197

IFU:	128610594 128610595 128610596 128610597 128610598 128610762

PHYP:

113524124 113524125

SMEO:

124387147

TFD:

113636597

TVC:

132849183

CGAR:

128527241 128528576

NGRA:

132883281 132889797

TRN:	134311801 134311802 134311803

AMEX:

103039627

CMAO:

118826322 118826323

EEE:	113582944 113590803

CHAR:

105900586

SPIL:

134072849 134072858 134072874 134072882 134102599

TRU:	101070868 101071841 101080232 115248012 115248013

TFS:	130538329 130538331 130538332 130539269

TNG:	GSTEN00016125G001 GSTEN00016134G001

LCO:	104917693 104924020

NCC:	104940932 104941058

TBEN:

117488443 117490710

CGOB:

115022806

PGEO:

117462682

GACU:

117558328

EMAC:

134866467

ELY:	117269563 117272242 117272243

EFO:	125898267 125901748

PLEP:

121939385 121952022

SLUC:

116045967

ECRA:

117954895

ESP:

116699759

PFLV:

114565114 114570013

GAT:

120816014

PPUG:

119206272

AFB:

129114977

CLUM:

117746545

PSWI:

130189685 130198009

CANA:

137782089

CVE:	137866048 137892792

MSAM:

119901973

SCHU:

122887207 122887208

CUD:

121519631

SPUL:

138604339 138612253

LMIX:

132979262

ALAT:

119011762

ASTR:

137612211

MZE:	101474730 106675939 106676194

ONL:	100693750 109195682 109195683 109195699

OAU:	116316201 116316225 120434437

OLA:

101157008

OML:

112147054

CSAI:

133452788

XMA:

102227191

XCO:

114147167

XHE:

116722321

PRET:

103479711

PFOR:

103135748

PLAI:

106948855

PMEI:

106914255

GAF:

122841414

PPRL:

129362291

CVG:

107081670

CTUL:

119794200

GMU:

124869592

NFU:

107383849

KMR:	108241650 108241654

ALIM:

106533255 106533266

NWH:

119414670

AOCE:

111568649 111568654

EJA:

138203057

BFRE:

138617308

MCEP:

125010344

CSEM:

103396384

POV:	109625444 109637167 109638123

SSEN:

122767875 122767883 122767892 122767898 122767900 122768649 122768658 122779511

SSOE:

131460065 131460672 131460678 131470973 131470982 131470987 131470988 131472551

HHIP:

117777688 117777689

HSP:	118117221 118118273

PPLT:

128453984 128454860 128454863 128454869 128454883 128455003

SMAU:

118310554 118311576

LCF:	108883349 108893535

SDU:

111231161

SLAL:

111653251

XGL:	120798539 120802087

HCQ:

109521313

SSCV:

125984634 125984635

SBIA:

133510826

PEE:

133411474

PTAO:

133488702

BPEC:

110154953 110159864

ENY:	140356617 140356618

BSPL:

114843905 114845031 114845073

SJO:

128368941

SSCO:

133990525

GMH:	115548416 115549671 115561466

SASA:

106569335 106599887 106599889

STRU:

115157201 115169574 115169575

OTW:	112226785 112226786 112260644

OMY:	110508572 110530492

OGO:	124010151 124040889 124041735

ONE:	115102943 115102944 115114571

OKI:	109872203 109872381 109875004

OKE:	118394819 118401954 127907637

OMM:	135512345 135558850 135558851

OCLA:

139376180 139387301

SALP:

111956862

SNH:	120019994 120020264 120055983

CCLU:

121535363 121581051 121581540

ELS:	105019795 105019804 106024913

OEP:

134036827

SFM:	108919335 108937621 114910968 114910986

PKI:	111845862 111845867

AANG:

118232872

AROT:

135261077

CCOG:

133137081 133137278 133137470

LOC:	102682983 102694619 102694818

PSPA:

121311421 121311422 121312642 121312643 121312849 121314286 121314287 121314288 121314311 121314312

ARUT:

117394306 117394549 117394961 117399933

PSEX:

120529851 120529852 120529853

LCM:	102357417 102361952

CMK:

103188298

RTP:

109923397

CPLA:

122550148

HOC:

132815858

LERI:

129714125

HAKA:

140732407(cyp8b1.1)

PMRN:

116946580 116946581 116946582 116946700

LRJ:	133353068 133353069 133353070

MMER:

123529448

RPHI:

132742682

MAEA:

128217726

E1B28_009492

» show all

Reference

1 [PMID:6766451]

Authors

Hansson R, Wikvall K.

Title

Hydroxylations in biosynthesis and metabolism of bile acids. Catalytic properties of different forms of cytochrome P-450.

Journal

J Biol Chem 255:1643-9 (1980)

Reference

2 [PMID:6811268]

Authors

Hansson R, Wikvall K.

Title

Hydroxylations in biosynthesis of bile acids. Cytochrome P-450 LM4 and 12alpha-hydroxylation of 5beta-cholestane-3alpha, 7alpha-diol.

Journal

Eur J Biochem 125:423-9 (1982)
DOI:10.1111/j.1432-1033.1982.tb06700.x

Reference

3 [PMID:1400444]

Authors

Ishida H, Noshiro M, Okuda K, Coon MJ

Title

Purification and characterization of 7 alpha-hydroxy-4-cholesten-3-one 12 alpha-hydroxylase.

Journal

J Biol Chem 267:21319-23 (1992)

Reference

4 [PMID:8943286]

Authors

Eggertsen G, Olin M, Andersson U, Ishida H, Kubota S, Hellman U, Okuda KI, Bjorkhem I.

Title

Molecular cloning and expression of rabbit sterol 12alpha-hydroxylase.

Journal

J Biol Chem 271:32269-75 (1996)
DOI:10.1074/jbc.271.50.32269

Sequence

[ocu:100009301]

Reference

5 [PMID:14643796]

Authors

Lundell K, Wikvall K.

Title

Gene structure of pig sterol 12alpha-hydroxylase (CYP8B1) and expression in fetal liver: comparison with expression of taurochenodeoxycholic acid 6alpha-hydroxylase (CYP4A21).

Journal

Biochim Biophys Acta 1634:86-96 (2003)
DOI:10.1016/j.bbalip.2003.09.002

Sequence

[ssc:403328]

Reference

6 [PMID:10747975]

Authors

del Castillo-Olivares A, Gil G.

Title

Alpha 1-fetoprotein transcription factor is required for the expression of sterol 12alpha -hydroxylase, the specific enzyme for cholic acid synthesis. Potential role in the bile acid-mediated regulation of gene transcription.

Journal

J Biol Chem 275:17793-9 (2000)
DOI:10.1074/jbc.M000996200

Reference

7 [PMID:12069850]

Authors

Yang Y, Zhang M, Eggertsen G, Chiang JY.

Title

On the mechanism of bile acid inhibition of rat sterol 12alpha-hydroxylase gene (CYP8B1) transcription: roles of alpha-fetoprotein transcription factor and hepatocyte nuclear factor 4alpha.

Journal

Biochim Biophys Acta 1583:63-73 (2002)
DOI:10.1016/S1388-1981(02)00186-5

Reference

8 [PMID:12543708]

Authors

Russell DW

Title

The enzymes, regulation, and genetics of bile acid synthesis.

Journal

Annu Rev Biochem 72:137-74 (2003)
DOI:10.1146/annurev.biochem.72.121801.161712

Sequence

[hsa:1582] [mmu:13124]

Reference

9 [PMID:30465713]

Authors

Fan L, Joseph JF, Durairaj P, Parr MK, Bureik M.

Title

Conversion of chenodeoxycholic acid to cholic acid by human CYP8B1.

Journal

Biol Chem 400:625-628 (2019)
DOI:10.1515/hsz-2018-0379

Other DBs

ExplorEnz - The Enzyme Database:

1.14.14.139

IUBMB Enzyme Nomenclature:

1.14.14.139

ExPASy - ENZYME nomenclature database:

1.14.14.139

BRENDA, the Enzyme Database:

1.14.14.139

All links

Gene (131)   
   MGENES (100)   
   RefGene (31)   
Protein sequence (17)   
   UniProt (5)   
   SWISS-PROT (4)   
   RefSeq(pep) (8)   
DNA sequence (9)   
   RefSeq(nuc) (9)   
Protein domain (5)   
   InterPro (5)   
All databases (162)   

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