KEGG ORTHOLOGY: K12405

ORTHOLOGY: K12405

Entry

K12405 KO

Name

HSD17B4

Definition

(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

ko00120

Primary bile acid biosynthesis

ko01040

Biosynthesis of unsaturated fatty acids

ko01100

Metabolic pathways

ko01212

Fatty acid metabolism

ko04146

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)

RRO:	104654581(HSD17B4)

RBB:	108517761 108529825(HSD17B4)

CJC:	100390100(HSD17B4)

SBQ:	101036776(HSD17B4)

MMU:	15488(Hsd17b4)

MCAL:

110284447(Hsd17b4)

MPAH:

110332881(Hsd17b4)

RNO:	79244(Hsd17b4)

MUN:	110539842 110558786

CGE:	103161104(Hsd17b4)

NGI:	103745113(Hsd17b4)

HGL:	101713520(Hsd17b4)

CCAN:

109674145 109689599 109689601

OCU:	100346781(HSD17B4)

TUP:	102468828(HSD17B4)

CFA:	474630(HSD17B4)

VVP:	112932403(HSD17B4)

AML:	100471749(HSD17B4)

UMR:	103670554(HSD17B4)

UAH:	113261629(HSD17B4)

ORO:	101385731(HSD17B4)

FCA:	101091840(HSD17B4)

PTG:	102968873(HSD17B4)

AJU:	106975089(HSD17B4)

BTA:	493643(HSD17B4)

BOM:	102268517(HSD17B4)

BIU:	109560984 109560985

BBUB:

102412917(HSD17B4)

PHD:	102329357(HSD17B4)

CHX:	102172142(HSD17B4)

OAS:	101114519(HSD17B4)

SSC:	397574(HSD17B4)

CFR:	102508317(HSD17B4)

CDK:	105104293(HSD17B4)

BACU:

103013794 103016570

LVE:	103084787(HSD17B4)

OOR:	101282963(HSD17B4)

DLE:	111183342(HSD17B4)

ECB:	100034046(HSD17B4)

EPZ:	103548315(HSD17B4)

EAI:	106833039(HSD17B4)

MYB:	102240475(HSD17B4)

MYD:	102767159(HSD17B4)

MNA:	107530713(HSD17B4)

HAI:	109374101(HSD17B4)

RSS:	109455167(HSD17B4)

DRO:	112315031(HSD17B4)

PALE:

102887000(HSD17B4)

LAV:	100659299(HSD17B4)

TMU:

101356123

MDO:	100028098(HSD17B4)

SHR:	100932771(HSD17B4)

PCW:	110202743(HSD17B4)

OAA:	100081754(HSD17B4)

GGA:	395785(HSD17B4)

MGP:	100545396(HSD17B4)

CJO:	107306670(HSD17B4)

APLA:

101791118(HSD17B4)

ACYG:

106048362(HSD17B4)

TGU:	100218226(HSD17B4)

LSR:	110479177(HSD17B4)

SCAN:

103821465(HSD17B4)

GFR:	102036502(HSD17B4)

FAB:	101807233(HSD17B4)

PHI:	102109547(HSD17B4)

PMAJ:

107216347(HSD17B4)

CCAE:

111941198(HSD17B4)

CCW:	104690497(HSD17B4)

ETL:	114056305(HSD17B4)

FPG:	101917400(HSD17B4)

FCH:	102058406(HSD17B4)

CLV:	102095771(HSD17B4)

EGZ:	104122491(HSD17B4)

NNI:	104014571(HSD17B4)

ACUN:

113489653(HSD17B4)

ASN:	102371766(HSD17B4)

AMJ:	102571005(HSD17B4)

PSS:	102451304(HSD17B4)

CMY:	102933522(HSD17B4)

CPIC:

101945395(HSD17B4)

ACS:	100558484(hsd17b4)

PVT:	110077461(SCP2D1) 110084344(HSD17B4)

PBI:	103059748(SCP2D1) 103063870(HSD17B4)

PMUR:

107294349(HSD17B4)

GJA:	107106522(SCP2D1) 107116225(HSD17B4)

XLA:	444492(hsd17b4.L)

XTR:	613082(hsd17b4)

NPR:	108787696(HSD17B4)

DRE:	393105(hsd17b4)

SRX:	107713552(hsd17b4) 107713572

SANH:

107703629(hsd17b4)

SGH:	107571774(hsd17b4)

IPU:	108265438(hsd17b4)

PHYP:

113529268(hsd17b4)

AMEX:

103046836(hsd17b4)

EEE:	113571049(hsd17b4)

TRU:	101062498(hsd17b4)

TNG:	GSTEN00030945G001

LCO:	104920820(hsd17b4)

NCC:	104966339(hsd17b4)

MZE:	101482787(hsd17b4)

OLA:	101159921(hsd17b4)

XMA:	102223774(hsd17b4)

XCO:	114154573(hsd17b4)

PRET:

103470095(hsd17b4)

NFU:	107394149(hsd17b4)

KMR:	108233704(hsd17b4)

CSEM:

103397642(hsd17b4)

LCF:	108882142(hsd17b4)

SDU:	111230866(hsd17b4)

HCQ:	109526033(hsd17b4)

BPEC:

110167024(hsd17b4)

MALB:

109956607(hsd17b4)

SASA:

106585849(hsd17b4)

OTW:	112247069(hsd17b4)

SALP:

111974039(hsd17b4)

ELS:	105014609(hsd17b4)

SFM:	108938133(hsd17b4)

PKI:	111856620(hsd17b4)

LCM:	102356548(HSD17B4)

CMK:	103181119(hsd17b4)

BFO:	BRAFLDRAFT_125736 BRAFLDRAFT_125738 BRAFLDRAFT_232676

CIN:

100187421

SPU:	581580(hsd17b4)

APLC:

110987677

SKO:	100367300 100367453

DME:	Dmel_CG3415(Mfe2)

DPO:	Dpse_GA17436

DAN:	Dana_GF19024

DER:

6550509

DPE:

6599365

DSI:	Dsimw501_GD29376(Dsim_GD29376)

DWI:

6648554

DYA:	Dyak_GE17252

DGR:	Dgri_GH12555

DMO:	Dmoj_GI11209

DAZ:

108619615

DNV:

108656784

DHE:

111598829

DVI:	Dvir_GJ18503

MDE:

101897498

AGA:	AgaP_AGAP008667

AAG:

5568633

CQU:	CpipJ_CPIJ001576

AME:

BIM:

BTER:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

HST:

DQU:

CFO:

LHU:

PGC:

OBO:

PCF:

NVI:

MDL:

TCA:	663655(17-beta-HD4)

DPA:

NVL:

BMOR:

PMAC:

PRAP:

HAW:

TNL:

PXY:

API:

DNX:

AGS:

CLEC:

PHU:	Phum_PHUM414590

ZNE:

110835121

FCD:	110842788 110850131 110850197 110860202 110860525

DPX:	DAPPUDRAFT_332860

ISC:	IscW_ISCW005296

TUT:

107364381

DPTE:

113789986

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

CBR:	CBG10899(Cbr-dhs-28) CBG13089(Cbr-maoc-1)

BMY:	Bm1_24050 Bm1_44740

LOA:	LOAG_00696 LOAG_08780

NAI:	NECAME_02432 NECAME_03406 NECAME_18174

HRO:	HELRODRAFT_98504

LGI:	LOTGIDRAFT_157973

PCAN:

CRG:

MYI:

OBI:

NVE:	NEMVE_v1g217427

EPA:

ADF:

PDAM:

SPIS:

HMG:	100208540 100209647

TAD:	TRIADDRAFT_19721

AQU:

100640903

FOX:	FOXG_17229

NHE:	NECHADRAFT_41481

PNO:	SNOG_03011

MBR:	MONBRDRAFT_23152

SRE:	PTSG_04583

DDI:	DDB_G0291247(mfeA)

DPP:	DICPUDRAFT_48962

DFA:	DFA_02002(mfeA)

ACAN:

ACA1_063620

TGO:	TGME49_234570(HAD2SCP2)

TET:	TTHERM_00317440

PTM:	GSPATT00021528001 GSPATT00022802001 GSPATT00029759001

SMIN:

v1.2.022875.t1(symbB.v1.2.022875.t1)

PIF:	PITG_02607 PITG_08421 PITG_19054

PSOJ:

PHYSODRAFT_284465 PHYSODRAFT_490678 PHYSODRAFT_538205

SPAR:

SPRG_00591 SPRG_12670 SPRG_16528 SPRG_19830

NGR:	NAEGRDRAFT_79710

CARE:

LT85_3600

AGE:	AA314_01285

LLU:	AKJ09_03288

MRM:	A7982_08830

CBOT:

ATE48_12745

PBF:	CFX0092_A1378

» 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)

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]

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