KEGG ORTHOLOGY: K10214

ORTHOLOGY: K10214

Entry

K10214                      KO

Symbol

ACOX2

Name

3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase [EC:1.17.99.3]

Pathway

map00120

Primary bile acid biosynthesis

map01100

Metabolic pathways

map03320

PPAR signaling pathway

map04146

Peroxisome

Module

M00104

Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate

M00862

beta-Oxidation, peroxisome, tri/dihydroxycholestanoyl-CoA => choloyl/chenodeoxycholoyl-CoA

Reaction

R08735

(25S)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA:acceptor 24,25-oxidoreductase

R08740

(25S)-3alpha,7alpha-dihydroxy-5beta-cholestanoyl-CoA:acceptor 24,25-oxidoreductase

Disease

H00628

Congenital bile acid synthesis defect

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09103 Lipid metabolism
   00120 Primary bile acid biosynthesis
    K10214  ACOX2; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
09140 Cellular Processes
  09141 Transport and catabolism
   04146 Peroxisome
    K10214  ACOX2; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
09150 Organismal Systems
  09152 Endocrine system
   03320 PPAR signaling pathway
    K10214  ACOX2; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
Enzymes [BR:ko01000]
1. Oxidoreductases
  1.17  Acting on CH or CH2 groups
   1.17.99  With unknown physiological acceptors
    1.17.99.3  3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
     K10214  ACOX2; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase

Other DBs

GO:

0033791

Genes

HSA:	8309(ACOX2)

PTR:	460479(ACOX2)

PPS:	100987633(ACOX2)

GGO:	101135855(ACOX2)

PON:	100173031(ACOX2)

PPYG:

129032448(ACOX2)

NLE:	100579193(ACOX2)

HMH:	116469171(ACOX2)

SSYN:

129479507(ACOX2)

MCC:	701565(ACOX2)

MCF:	102142571(ACOX2)

MTHB:

126948824

MNI:	105482947(ACOX2)

CSAB:

103227815(ACOX2)

CATY:

105585630(ACOX2)

PANU:

101010658(ACOX2)

TGE:	112618624(ACOX2)

MLEU:

105552659(ACOX2)

RRO:	104660198(ACOX2)

RBB:	108543617(ACOX2)

TFN:	117083061(ACOX2)

PTEH:

111532709(ACOX2)

CANG:

105515406(ACOX2)

CJC:	100404475(ACOX2)

SBQ:	101051802(ACOX2)

CIMI:

108303497(ACOX2)

ANAN:

105724318(ACOX2)

CSYR:

103260875(ACOX2)

MMUR:

105884922(ACOX2)

LCAT:

123623526(ACOX2)

PCOQ:

105805735(ACOX2)

OGA:	100958565(ACOX2)

MMU:	93732(Acox2)

MCAL:

110309300(Acox2)

MPAH:

110325739(Acox2)

RNO:	252898(Acox2)

MCOC:

116073384(Acox2)

ANU:	117706119(Acox2)

MUN:	110554615(Acox2)

CGE:	100766043(Acox2)

MAUA:

101831411(Acox2)

PROB:

127238208(Acox2)

PLEU:

114680724(Acox2)

MORG:

121455134(Acox2)

MFOT:

126513015

AAMP:

119827884(Acox2)

NGI:	103740540(Acox2)

HGL:	101716015(Acox2)

CPOC:

100730978(Acox2)

CCAN:

109697261(Acox2)

DORD:

105981391(Acox2)

DSP:	122107967(Acox2)

PLOP:

125358690(Acox2)

NCAR:

124969525

MMMA:

107149659(Acox2)

OCU:	100009549(ACOX2)

OPI:	101527454(ACOX2)

TUP:	102491187(ACOX2)

GVR:	103593550(ACOX2)

CFA:	100855488(ACOX2)

CLUD:

112665973(ACOX2)

VVP:	112909557(ACOX2)

VLG:	121495008(ACOX2)

NPO:	129521839(ACOX2)

AML:	100483316(ACOX2)

UMR:	103674276(ACOX2)

UAH:	113257024(ACOX2)

UAR:	123798800(ACOX2)

ELK:

111144181

LLV:

125078328

MPUF:

101687162(ACOX2)

MNP:	132011451(ACOX2)

MLK:	131824071(ACOX2)

NVS:	122909308(ACOX2)

ORO:	101386426(ACOX2)

EJU:	114214660(ACOX2)

ZCA:	113916645(ACOX2)

MLX:	118005730(ACOX2)

NSU:	110584818(ACOX2)

LWW:	102749711(ACOX2)

FCA:	101101039(ACOX2)

PYU:	121016789(ACOX2)

PCOO:

112852623(ACOX2)

PBG:	122487375(ACOX2)

PVIV:

125158271(ACOX2)

LRUF:

124524999

PTG:	102972085(ACOX2)

PPAD:

109279078(ACOX2)

PUC:

125929528

AJU:

106968832

HHV:	120238645(ACOX2)

BTA:	514969(ACOX2)

BOM:	102287053(ACOX2)

BIU:	109576197(ACOX2)

BBUB:

102404658(ACOX2)

BBIS:

104997077(ACOX2)

CHX:	102184385(ACOX2)

OAS:	101121460(ACOX2)

BTAX:

128044329(ACOX2)

CSUM:

138087216(ACOX2)

ODA:	120867983(ACOX2)

CCAD:

122424314(ACOX2)

MREE:

136166717(ACOX2)

MBEZ:

129547971(ACOX2)

SSC:	100516877(ACOX2)

CFR:	102512694(ACOX2)

CBAI:

105079554(ACOX2)

CDK:	105089810(ACOX2)

VPC:	102527334(ACOX2)

BACU:

102999785(ACOX2)

BMUS:

118903835(ACOX2)

LVE:	103086040(ACOX2)

OOR:	101288466(ACOX2)

DLE:	111174239(ACOX2)

PCAD:

102973306(ACOX2)

PSIU:

116762734(ACOX2)

NASI:

112400617(ACOX2)

ECB:	100050425(ACOX2)

EPZ:	103550743(ACOX2)

EAI:	106848667(ACOX2)

MYB:	102260512(ACOX2)

MYD:	102766328(ACOX2)

MMYO:

118667935(ACOX2)

MLF:	102428739(ACOX2)

MDT:	132215244(ACOX2)

PKL:	118715071(ACOX2)

EFUS:

103290449(ACOX2)

MNA:	107531454(ACOX2)

DRO:	112301264(ACOX2)

SHON:

118983389(ACOX2)

AJM:	119043406(ACOX2)

PDIC:

114501728(ACOX2)

PHAS:

123814456(ACOX2)

MMF:	118633599(ACOX2)

PPAM:

129088225(ACOX2)

HAI:	109388232(ACOX2)

RFQ:	117036966(ACOX2)

PALE:

102884254(ACOX2)

PGIG:

120599535(ACOX2)

PVP:	105299837(ACOX2)

RAY:	107503398(ACOX2)

MJV:	108394908(ACOX2)

TOD:	119230556(ACOX2)

SARA:

101557002(ACOX2)

SETR:

126013205(ACOX2)

LAV:	100655728(ACOX2)

TMU:

101353097

ETF:	101638572(ACOX2)

DNM:	101442926(ACOX2)

MDO:	100028782(ACOX2)

GAS:	123246882(ACOX2)

SHR:	100924634(ACOX2)

AFZ:

127550706

PCW:	110195963(ACOX2)

TVP:	118831233(ACOX2)

PBRV:

138158170(ACOX2)

OAA:	100074731(ACOX2)

GGA:	416068(ACOX2)

PCOC:

116237336(ACOX2)

MGP:	100545690(ACOX2)

CJO:	107319785(ACOX2)

TPAI:

128078692(ACOX2)

LMUT:

125698947(ACOX2)

NMEL:

110404665(ACOX2)

APLA:

101804343(ACOX2)

ACYG:

106035476(ACOX2)

CATA:

118248178(ACOX2)

AFUL:

116493226(ACOX2)

TGU:	100228411(ACOX2)

LSR:	110470870(ACOX2)

SCAN:

103817065(ACOX2)

PMOA:

120514182(ACOX2)

OTC:	121347290(ACOX2)

PRUF:

121350699(ACOX2)

GFR:	102044390(ACOX2)

FAB:	101820029(ACOX2)

OMA:	130258427(ACOX2)

PHI:	102102070(ACOX2)

PMAJ:

107210348(ACOX2)

CCAE:

111935016(ACOX2)

CCW:	104683332(ACOX2)

CBRC:

103614344(ACOX2)

ETL:	114056799(ACOX2)

ZAB:	102066854(ACOX2)

ZLE:	135453279(ACOX2)

ACHL:

103802722(ACOX2)

SVG:	106851091(ACOX2)

MMEA:

130589161(ACOX2)

HRT:	120758395(ACOX2)

SATI:

136365867(ACOX2)

FPG:	101910731(ACOX2)

FCH:	102048175(ACOX2)

CCRI:

104154848(ACOX2)

NNT:	104403304(ACOX2)

SHAB:

115614772(ACOX2)

ACUN:

113484962(ACOX2)

TALA:

104357214(ACOX2)

ACHC:

115353747(ACOX2)

HALD:

104323861(ACOX2)

HLE:	104833984(ACOX2)

AGEN:

126049759

GCL:

127021570

CSTI:

104549918(ACOX2)

LDI:	104349688(ACOX2)

MNB:	103769714(ACOX2)

DPUB:

104305103(ACOX2)

PPUS:

135182626(ACOX2)

AVIT:

104272361(ACOX2)

BRHI:

104496268(ACOX2)

EGZ:	104134791(ACOX2)

NNI:	104016906(ACOX2)

PCRI:

104034223(ACOX2)

PCAO:

104041431(ACOX2)

PADL:

103918901(ACOX2)

AFOR:

103903237(ACOX2)

FGA:	104076315(ACOX2)

GSTE:

104260140(ACOX2)

CLV:	102092775(ACOX2)

MUI:	104533437(ACOX2)

PGUU:

104465920(ACOX2)

PLET:

104628479(ACOX2)

EHS:	104516442(ACOX2)

CMAC:

104476997(ACOX2)

CUCA:

104064609(ACOX2)

TEO:	104378997(ACOX2)

BREG:

104632277(ACOX2)

OHA:	104331333(ACOX2)

ACAR:

104532473(ACOX2)

CPEA:

104388179(ACOX2)

CVF:	104293064(ACOX2)

RTD:	128915531(ACOX2)

AAM:	106489663(ACOX2)

AROW:

112976606(ACOX2)

NPD:	112958598(ACOX2)

TGT:	104580279(ACOX2)

DNE:	112984663(ACOX2)

SCAM:

104144689(ACOX2)

ASN:	102380977(ACOX2)

AMJ:	102563802(ACOX2)

CPOO:

109311219(ACOX2)

GGN:	109289012(ACOX2)

PSS:	102450027(ACOX2)

CMY:	102935400(ACOX2)

CCAY:

125640597(ACOX2)

DCC:	119858606(ACOX2)

CPIC:

101950611(ACOX2)

TST:	117879967(ACOX2)

CABI:

116825650(ACOX2)

MRV:	120368668(ACOX2)

ACS:	100556696(acox2)

ASAO:

132769437(ACOX2)

PVT:	110079915(ACOX2)

SUND:

121920729 121920730 121924949

PBI:	103062545(ACOX2)

PMUR:

107296584(ACOX2)

CTIG:

120300493(ACOX2)

TSR:	106550842(ACOX2)

PGUT:

117660659(ACOX2)

APRI:

131190239(ACOX2)

PTEX:

113450449(ACOX2)

NSS:	113422110(ACOX2)

VKO:	123034601(ACOX2)

PMUA:

114591888(ACOX2)

PRAF:

128408517(ACOX2)

ZVI:	118080857(ACOX2)

HCG:	128344586(ACOX2)

GJA:	107119599(ACOX2)

STOW:

125429067 125429068

EMC:	129326958 129329209

XLA:	414480(acox2.L)

XTR:	548740(acox2)

NPR:	108791853(ACOX2)

RTEM:

120945173(ACOX2)

BBUF:

120979104 120979105 120979106

BGAR:

122943473 122943873

MUO:	115472392(ACOX2)

GSH:	117351297(ACOX2)

LCM:	102363316(ACOX2)

CMK:

103179753

RTP:

109920851

CPLA:

122559256(kctd6a)

HOC:

LERI:

CIN:

SCLV:

Reference

PMID:9218493

Authors

Pedersen JI, Eggertsen G, Hellman U, Andersson U, Bjorkhem I

Title

Molecular cloning and expression of cDNA encoding 3alpha,7alpha,12alpha-trihydroxy-5beta-chole stanoyl-CoA oxidase from rabbit liver.

Journal

J Biol Chem 272:18481-9 (1997)
DOI:10.1074/jbc.272.29.18481

Sequence

[ocu:100009549]

Reference

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

LinkDB

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