KEGG   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)
RRO: 104654581(HSD17B4)
RBB: 108529825(HSD17B4)
TFN: 117065971(HSD17B4)
PTEH: 111530208(HSD17B4)
CJC: 100390100(HSD17B4)
SBQ: 101036776(HSD17B4)
MMUR: 105872240
MMU: 15488(Hsd17b4)
MCAL: 110284447(Hsd17b4)
MPAH: 110332881(Hsd17b4)
RNO: 79244(Hsd17b4)
MCOC: 116087150(Hsd17b4)
CGE: 103161104(Hsd17b4)
PLEU: 114694768(Hsd17b4)
NGI: 103745113(Hsd17b4)
HGL: 101713520(Hsd17b4)
CCAN: 109674145
OCU: 100346781(HSD17B4)
TUP: 102468828(HSD17B4)
CFA: 474630(HSD17B4)
VVP: 112932403(HSD17B4)
VLG: 121494637(HSD17B4)
AML: 100471749(HSD17B4)
UMR: 103670554(HSD17B4)
UAH: 113261629(HSD17B4)
ORO: 101385731(HSD17B4)
ELK: 111158891
MPUF: 101683185(HSD17B4)
EJU: 114209435(HSD17B4)
MLX: 118016560(HSD17B4)
FCA: 101091840(HSD17B4)
PYU: 121010581(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)
LVE: 103084787(HSD17B4)
OOR: 101282963(HSD17B4)
DLE: 111183342(HSD17B4)
PCAD: 102996127(HSD17B4)
ECB: 100034046(HSD17B4)
EPZ: 103548315(HSD17B4)
EAI: 106833039(HSD17B4)
MYB: 102240475(HSD17B4)
MYD: 102767159(HSD17B4)
MMYO: 118656167(HSD17B4)
MNA: 107530713(HSD17B4)
HAI: 109374101(HSD17B4)
DRO: 112315031(HSD17B4)
SHON: 118975196(HSD17B4)
AJM: 119051192(HSD17B4)
MMF: 118642891(HSD17B4)
PGIG: 120590862(HSD17B4)
TOD: 119243559(HSD17B4)
LAV: 100659299(HSD17B4)
TMU: 101356123
MDO: 100028098(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)
TGU: 100218226(HSD17B4)
LSR: 110479177(HSD17B4)
SCAN: 103821465(HSD17B4)
PMOA: 120512524(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)
PADL: 103912347(HSD17B4)
NPD: 112958912(HSD17B4)
ASN: 102371766(HSD17B4)
AMJ: 102571005(HSD17B4)
CPOO: 109323436(HSD17B4)
GGN: 109297318(HSD17B4)
PSS: 102451304(HSD17B4)
CPIC: 101945395(HSD17B4)
TST: 117879364(HSD17B4)
CABI: 116836212(HSD17B4)
ACS: 100558484(hsd17b4)
PVT: 110084344(HSD17B4)
PBI: 103063870(HSD17B4)
PMUR: 107294349(HSD17B4)
TSR: 106543382(HSD17B4)
PGUT: 117674677(HSD17B4)
PMUA: 114606393(HSD17B4)
ZVI: 118076708(HSD17B4)
GJA: 107116225(HSD17B4)
XLA: 444492(hsd17b4.L)
XTR: 613082(hsd17b4)
NPR: 108787696(HSD17B4)
DRE: 393105(hsd17b4)
SANH: 107703629(hsd17b4)
SGH: 107571774(hsd17b4)
IPU: 108265438(hsd17b4)
PHYP: 113529268(hsd17b4)
AMEX: 103046836(hsd17b4)
EEE: 113571049(hsd17b4)
TRU: 101062498(hsd17b4)
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)
MSAM: 119891683(hsd17b4)
CUD: 121509730(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)
CVG: 107086311(hsd17b4)
CTUL: 119798579(hsd17b4)
NFU: 107394149(hsd17b4)
KMR: 108233704(hsd17b4)
ALIM: 106513677(hsd17b4)
AOCE: 111564685(hsd17b4)
POV: 109637138(hsd17b4)
LCF: 108882142(hsd17b4)
SDU: 111230866(hsd17b4)
SLAL: 111645149(hsd17b4)
XGL: 120805559(hsd17b4)
HCQ: 109526033(hsd17b4)
BPEC: 110167024(hsd17b4)
MALB: 109956607(hsd17b4)
SASA: 106585849(hsd17b4)
OTW: 112247069(hsd17b4)
OMY: 110525264(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
LCM: 102356548(HSD17B4)
CMK: 103181119(hsd17b4)
BFO: 118409354
BBEL: 109480804
CIN: 100187421
SCLV: 120338122
SPU: 581580
DME: Dmel_CG3415(Mfe2)
DMN: 108161558
DGR: 6565453
CCAT: 101461807
ACOZ: 120955687
AARA: 120901803
AAG: 5568633
AALB: 109428196
CPII: 120414661
AME: 409986
ACER: 107999187
BBIF: 117204816
NVI: 100123018
CCIN: 107263397
TCA: 663655(17-beta-HD4)
AGB: 108915760
LDC: 111505860
APLN: 108736403
OTU: 111423793
BMOR: 101745499
API: 100167575
DNX: 107163428
AGS: 114120233
RMD: 113555196
HHAL: 106683228
NLU: 111057072
CSEC: 111873091
DMK: 116931070
HAME: 121875772
HAZT: 108681612
EAF: 111696524
DSV: 119440587
RSAN: 119387719
VDE: 111252755
VJA: 111266535
SDM: 118180496
CEL: CELE_E04F6.3(maoc-1) CELE_M03A8.1(dhs-28)
BGT: 106065027
GAE: 121385566
PMAX: 117334218
NVE: 5504006
ATEN: 116294223
DDI: DDB_G0291247(mfeA)
 » 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
  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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