KEGG ORTHOLOGY: K12405

ORTHOLOGY: K12405

Entry

K12405                      KO

Symbol

HSD17B4

Name

(3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2 [EC:1.1.1.- 4.2.1.107 4.2.1.119]

Pathway

map00120

Primary bile acid biosynthesis

map01040

Biosynthesis of unsaturated fatty acids

map01100

Metabolic pathways

map01212

Fatty acid metabolism

map04146

Peroxisome

Module

M00104

Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate

M00861

beta-Oxidation, peroxisome, VLCFA

M00862

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

Disease

H00407

Peroxisomal beta-oxidation enzyme deficiency

H02095

Perrault syndrome

H02098

D-bifunctional protein deficiency

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09103 Lipid metabolism
   00120 Primary bile acid biosynthesis
    K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2
   01040 Biosynthesis of unsaturated fatty acids
    K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2
09140 Cellular Processes
  09141 Transport and catabolism
   04146 Peroxisome
    K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2
Enzymes [BR:ko01000]
1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.-
     K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2
4. Lyases
  4.2  Carbon-oxygen lyases
   4.2.1  Hydro-lyases
    4.2.1.107  3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase
     K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2
    4.2.1.119  enoyl-CoA hydratase 2
     K12405  HSD17B4; (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2

Other DBs

RN:	R04809 R04810 R04812 R04813 R07935 R07936 R07951 R07952 R09698

GO:

0033989

Genes

HSA:	3295(HSD17B4)

PTR:	462019(HSD17B4)

PPS:	100981028(HSD17B4)

GGO:	101129538(HSD17B4)

PON:	100448539(HSD17B4)

NLE:	100590035(HSD17B4)

MCC:	698369(HSD17B4)

MCF:	102123675(HSD17B4)

CSAB:

103244395(HSD17B4)

CATY:

105590649(HSD17B4)

PANU:

101004327(HSD17B4)

TGE:	112625827(HSD17B4)

RRO:	104654581(HSD17B4)

RBB:	108529825(HSD17B4)

TFN:	117065971(HSD17B4)

PTEH:

111530208(HSD17B4)

CJC:	100390100(HSD17B4)

SBQ:	101036776(HSD17B4)

CSYR:

103262046

MMUR:

105872240

OGA:	100966879(HSD17B4)

MMU:	15488(Hsd17b4)

MCAL:

110284447(Hsd17b4)

MPAH:

110332881(Hsd17b4)

RNO:	79244(Hsd17b4)

MCOC:

116087150(Hsd17b4)

CGE:	103161104(Hsd17b4)

MAUA:

101827399(Hsd17b4)

PLEU:

114694768(Hsd17b4)

MORG:

121441957 121453615(Hsd17b4)

NGI:	103745113(Hsd17b4)

HGL:	101713520(Hsd17b4)

CPOC:

100379226(Hsd17b4)

CCAN:

109674145

DORD:

105985471(Hsd17b4)

DSP:	122101973(Hsd17b4)

NCAR:

124986751

OCU:	100346781(HSD17B4)

OPI:	101530287(HSD17B4)

TUP:	102468828(HSD17B4)

CFA:	474630(HSD17B4)

VVP:	112932403(HSD17B4)

VLG:	121494637(HSD17B4)

AML:	100471749(HSD17B4)

UMR:	103670554(HSD17B4)

UAH:	113261629(HSD17B4)

UAR:	123790430(HSD17B4)

ELK:

111158891

LLV:

125100000

MPUF:

101683185(HSD17B4)

ORO:	101385731(HSD17B4)

EJU:	114209435(HSD17B4)

ZCA:	113928790(HSD17B4)

MLX:	118016560(HSD17B4)

FCA:	101091840(HSD17B4)

PYU:	121010581(HSD17B4)

PBG:	122486641(HSD17B4)

PTG:	102968873(HSD17B4)

PPAD:

109278892(HSD17B4)

AJU:	106975089(HSD17B4)

HHV:	120240242(HSD17B4)

BTA:	493643(HSD17B4)

BOM:	102268517(HSD17B4)

BBUB:

102412917(HSD17B4)

CHX:	102172142(HSD17B4)

OAS:	101114519(HSD17B4)

ODA:	120864671(HSD17B4)

CCAD:

122440074(HSD17B4)

SSC:	397574(HSD17B4)

CFR:	102508317(HSD17B4)

CBAI:

105082053(HSD17B4)

CDK:	105104293(HSD17B4)

VPC:	102537534(HSD17B4)

LVE:	103084787(HSD17B4)

OOR:	101282963(HSD17B4)

DLE:	111183342(HSD17B4)

PCAD:

102996127(HSD17B4)

PSIU:

116751686(HSD17B4)

ECB:	100034046(HSD17B4)

EPZ:	103548315(HSD17B4)

EAI:	106833039(HSD17B4)

MYB:	102240475(HSD17B4)

MYD:	102767159(HSD17B4)

MMYO:

118656167(HSD17B4)

MLF:	102425700(HSD17B4)

MNA:	107530713(HSD17B4)

PKL:	118703106(HSD17B4)

HAI:	109374101(HSD17B4)

DRO:	112315031(HSD17B4)

SHON:

118975196(HSD17B4)

AJM:	119051192(HSD17B4)

PDIC:

114494306(HSD17B4)

PHAS:

123818113(HSD17B4)

MMF:	118642891(HSD17B4)

RFQ:	117024890(HSD17B4)

PALE:

102887000(HSD17B4)

PGIG:

120590862(HSD17B4)

PVP:	105290860(HSD17B4)

RAY:	107511154(HSD17B4)

TOD:	119243559(HSD17B4)

SARA:

101542842(HSD17B4)

LAV:	100659299(HSD17B4)

TMU:

101356123

DNM:	101423884(HSD17B4)

MDO:	100028098(HSD17B4)

GAS:	123235791(HSD17B4)

SHR:	100932771(HSD17B4)

PCW:	110202743(HSD17B4)

OAA:	100081754(HSD17B4)

GGA:	395785(HSD17B4)

PCOC:

116234067(HSD17B4)

CJO:	107306670(HSD17B4)

NMEL:

110389447(HSD17B4)

APLA:

101791118(HSD17B4)

ACYG:

106048362(HSD17B4)

AFUL:

116500754(HSD17B4)

TGU:	100218226(HSD17B4)

LSR:	110479177(HSD17B4)

SCAN:

103821465(HSD17B4)

PMOA:

120512524(HSD17B4)

OTC:	121332706(HSD17B4)

PRUF:

121362562(HSD17B4)

GFR:	102036502(HSD17B4)

FAB:	101807233(HSD17B4)

PHI:	102109547(HSD17B4)

PMAJ:

107216347(HSD17B4)

CCAE:

111941198(HSD17B4)

CCW:	104690497(HSD17B4)

ETL:	114056305(HSD17B4)

ZAB:	102062238(HSD17B4)

FPG:	101917400(HSD17B4)

FCH:	102058406(HSD17B4)

CLV:	102095771(HSD17B4)

EGZ:	104122491(HSD17B4)

NNI:	104014571(HSD17B4)

ACUN:

113489653(HSD17B4)

TALA:

104363287(HSD17B4)

PADL:

103912347(HSD17B4)

ACHC:

115336530(HSD17B4)

AROW:

112978041(HSD17B4)

NPD:	112958912(HSD17B4)

DNE:

112992405

ASN:	102371766(HSD17B4)

AMJ:	102571005(HSD17B4)

CPOO:

109323436(HSD17B4)

GGN:	109297318(HSD17B4)

PSS:	102451304(HSD17B4)

CPIC:

101945395(HSD17B4)

TST:	117879364(HSD17B4)

CABI:

116836212(HSD17B4)

MRV:	120408024(HSD17B4)

ACS:	100558484(hsd17b4)

PVT:	110084344(HSD17B4)

SUND:

121921111(HSD17B4)

PBI:	103063870(HSD17B4)

PMUR:

107294349(HSD17B4)

TSR:	106543382(HSD17B4)

PGUT:

117674677(HSD17B4)

VKO:	123031365(HSD17B4)

PMUA:

114606393(HSD17B4)

ZVI:	118076708(HSD17B4)

GJA:	107116225(HSD17B4)

STOW:

125436558(HSD17B4)

XLA:	444492(hsd17b4.L)

XTR:	613082(hsd17b4)

NPR:	108787696(HSD17B4)

RTEM:

120931986(HSD17B4)

BBUF:

120992600(HSD17B4)

BGAR:

122924617(HSD17B4)

DRE:	393105(hsd17b4)

SANH:

107703629(hsd17b4)

SGH:	107571774(hsd17b4)

CAUA:

113094195 113106903

PPRM:

120475505(hsd17b4)

MAMB:

125279588(hsd17b4)

IPU:	108265438(hsd17b4)

PHYP:

113529268(hsd17b4)

SMEO:

124399595(hsd17b4)

TFD:	113656214(hsd17b4)

AMEX:

103046836(hsd17b4)

EEE:	113571049(hsd17b4)

TRU:	101062498(hsd17b4)

TNG:	GSTEN00030945G001

LCO:	104920820(hsd17b4)

NCC:	104966339(hsd17b4)

CGOB:

115013548(hsd17b4)

ELY:	117252434(hsd17b4)

PLEP:

121944362(hsd17b4)

SLUC:

116054060(hsd17b4)

ECRA:

117944965(hsd17b4)

PFLV:

114555481(hsd17b4)

GAT:	120830783(hsd17b4)

PPUG:

119225552(hsd17b4)

MSAM:

119891683(hsd17b4)

CUD:	121509730(hsd17b4)

ALAT:

119019193(hsd17b4)

MZE:	101482787(hsd17b4)

ONL:	100706762(hsd17b4)

OAU:	116310500(hsd17b4)

OLA:	101159921(hsd17b4)

OML:	112148954(hsd17b4)

XMA:	102223774(hsd17b4)

XCO:	114154573(hsd17b4)

XHE:	116730397(hsd17b4)

PRET:

103470095(hsd17b4)

PFOR:

103141760(hsd17b4)

PLAI:

106944940(hsd17b4)

PMEI:

106923064(hsd17b4)

GAF:	122827419(hsd17b4)

CVG:	107086311(hsd17b4)

CTUL:

119798579(hsd17b4)

GMU:	124877861(hsd17b4)

NFU:	107394149(hsd17b4)

KMR:	108233704(hsd17b4)

ALIM:

106513677(hsd17b4)

NWH:	119413904(hsd17b4)

AOCE:

111564685(hsd17b4)

POV:	109637138(hsd17b4)

SSEN:

122769476(hsd17b4)

HHIP:

117768550(hsd17b4)

HSP:	118114529(hsd17b4)

LCF:	108882142(hsd17b4)

SDU:	111230866(hsd17b4)

SLAL:

111645149(hsd17b4)

XGL:	120805559(hsd17b4)

HCQ:	109526033(hsd17b4)

BPEC:

110167024(hsd17b4)

MALB:

109956607(hsd17b4)

BSPL:

114861822(hsd17b4)

SASA:

106585849(hsd17b4)

OTW:	112247069(hsd17b4)

OMY:	110525264(hsd17b4)

OGO:	124034633(hsd17b4)

ONE:	115111275(hsd17b4)

SALP:

111974039(hsd17b4)

SNH:	120045172(hsd17b4)

ELS:	105014609(hsd17b4)

SFM:	108938133(hsd17b4)

PKI:	111856620(hsd17b4)

AANG:

118206736(hsd17b4)

LOC:	102697531(hsd17b4)

PSPA:

121302596(hsd17b4) 121316706

ARUT:

117427495 117973220

LCM:	102356548(HSD17B4)

CMK:	103181119(hsd17b4)

BFO:

BBEL:

CIN:

SCLV:

SPU:

DME:	Dmel_CG3415(Mfe2)

DPO:	Dpse_GA17436

DMN:

DGR:

LSQ:

HIS:

AGA:	AgaP_AGAP008667

ACOZ:

AARA:

ASTE:

AAG:

AALB:

CQU:	CpipJ_CPIJ001576

CPII:

120414661

CNS:	116337519 116349688

BCOO:

NVI:

CGLO:

FAS:

DAM:

AGIF:

TCA:	663655(17-beta-HD4)

SOY:

CSET:

AGB:

LDC:

APLN:

PPYR:

116158989 116159225 116159796 116159821 116176974 116179454

OTU:

BMOR:

API:

DNX:

AGS:

RMD:

DCI:	103506752 108253294

HHAL:

NLU:

FOC:

TPAL:

CSEC:

DPX:	DAPPUDRAFT_332860

DMK:

PVM:

PJA:

PCHN:

HAME:

PCLA:

PTRU:

HAZT:

EAF:

LSM:

PPOI:

DSV:

RSAN:

VDE:

VJA:

DFR:	124493442 124496269

SDM:

118180496

CEL:	CELE_E04F6.3(maoc-1) CELE_M03A8.1(dhs-28)

CBR:	CBG_10899(Cbr-dhs-28)

BGT:

GAE:

HRF:

HRJ:

CRG:

MYI:

PMAX:

MMER:

OSN:

NVE:

ATEN:

XEN:

TAD:	TRIADDRAFT_19721

MBR:	MONBRDRAFT_23152

DDI:	DDB_G0291247(mfeA)

TET:	TTHERM_00317440

PCAY:

FRD00_09620

» show all

Reference

PMID:9089413

Authors

Jiang LL, Miyazawa S, Souri M, Hashimoto T

Title

Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein.

Journal

J Biochem 121:364-9 (1997)
DOI:10.1093/oxfordjournals.jbchem.a021596

Sequence

[hsa:3295]

Reference

PMID:15644212

Authors

Koski KM, Haapalainen AM, Hiltunen JK, Glumoff T

Title

Crystal structure of 2-enoyl-CoA hydratase 2 from human peroxisomal multifunctional enzyme type 2.

Journal

J Mol Biol 345:1157-69 (2005)
DOI:10.1016/j.jmb.2004.11.009

Sequence

[hsa:3295]

LinkDB

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