KEGG   ORTHOLOGY: K07515
Entry
K07515                      KO                                     

Name
HADHA
Definition
enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:4.2.1.17 1.1.1.211]
Pathway
ko00062  Fatty acid elongation
ko00071  Fatty acid degradation
ko00280  Valine, leucine and isoleucine degradation
ko00310  Lysine degradation
ko00380  Tryptophan metabolism
ko00410  beta-Alanine metabolism
ko00627  Aminobenzoate degradation
ko00640  Propanoate metabolism
ko00650  Butanoate metabolism
ko00930  Caprolactam degradation
ko01100  Metabolic pathways
ko01110  Biosynthesis of secondary metabolites
ko01120  Microbial metabolism in diverse environments
ko01200  Carbon metabolism
ko01212  Fatty acid metabolism
Module
M00032  Lysine degradation, lysine => saccharopine => acetoacetyl-CoA
M00085  Fatty acid elongation in mitochondria
M00087  beta-Oxidation
Disease
H00489  LCHAD deficiency
H00525  Disorders of mitochondrial fatty-acid oxidation
H01352  Mitochondrial trifunctional protein deficiency
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00640 Propanoate metabolism
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
   00650 Butanoate metabolism
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
  09103 Lipid metabolism
   00062 Fatty acid elongation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
   00071 Fatty acid degradation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
   00310 Lysine degradation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
   00380 Tryptophan metabolism
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
  09106 Metabolism of other amino acids
   00410 beta-Alanine metabolism
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
  09111 Xenobiotics biodegradation and metabolism
   00627 Aminobenzoate degradation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
   00930 Caprolactam degradation
    K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
Enzymes [BR:ko01000]
 1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.211  long-chain-3-hydroxyacyl-CoA dehydrogenase
     K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
 4. Lyases
  4.2  Carbon-oxygen lyases
   4.2.1  Hydro-lyases
    4.2.1.17  enoyl-CoA hydratase
     K07515  HADHA; enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
Other DBs
RN: R01778 R02685 R03026 R03045 R04137 R04170 R04204 R04224 R04737 R04738 R04739 R04740 R04741 R04743 R04744 R04745 R04746 R04748 R04749 R05595 R06942
GO: 0004300 0016509
Genes
HSA: 3030(HADHA)
PTR: 459079(HADHA)
PPS: 100973698 100978217(HADHA)
GGO: 101143053(HADHA)
PON: 100172963(HADHA)
NLE: 100593214 100593233(HADHA)
MCC: 695975(HADHA)
MCF: 102142372(HADHA)
CSAB: 103220642(HADHA)
RRO: 104677892(HADHA)
RBB: 108532679(HADHA)
CJC: 100390439(HADHA)
SBQ: 101047074(HADHA)
MMU: 97212(Hadha)
MCAL: 110294690(Hadha)
MPAH: 110324206(Hadha)
RNO: 170670(Hadha)
MUN: 110547680(Hadha)
CGE: 100757947(Hadha)
NGI: 103735655(Hadha)
HGL: 101724137(Hadha)
CCAN: 109685831(Hadha)
OCU: 100355022(HADHA)
TUP: 102471941(HADHA) 102478660
CFA: 100856745(HADHA)
VVP: 112908532(HADHA)
AML: 100469654(HADHA)
UMR: 103671672(HADHA)
UAH: 113270772(HADHA)
ORO: 101364854(HADHA)
ELK: 111154088
FCA: 101084701(HADHA)
PTG: 102966470(HADHA)
PPAD: 109267284(HADHA)
AJU: 106967830(HADHA)
BTA: 281810(HADHA)
BOM: 102287426(HADHA)
BIU: 109566315(HADHA)
BBUB: 102398211(HADHA)
CHX: 102189046(HADHA)
OAS: 100192316(HADHA)
SSC: 397012(HADHA)
CFR: 102516648(HADHA)
CDK: 105098509(HADHA)
BACU: 103001885(HADHA)
LVE: 103086620(HADHA)
OOR: 101286957(HADHA)
DLE: 111174988(HADHA)
PCAD: 102996843(HADHA)
ECB: 100071381(HADHA)
EPZ: 103546462(HADHA)
EAI: 106840921(HADHA)
MYB: 102263028(HADHA)
MYD: 102751724(HADHA)
MNA: 107534200(HADHA)
HAI: 109383051(HADHA)
DRO: 112297764(HADHA)
PALE: 102889313(HADHA)
RAY: 107498535(HADHA)
MJV: 108392159(HADHA)
LAV: 100675857(HADHA)
TMU: 101353485
MDO: 100030776(HADHA)
SHR: 100928252(HADHA)
PCW: 110206510(HADHA)
OAA: 100077524(HADHA)
GGA: 395929(HADHA)
MGP: 100548080(HADHA)
CJO: 107312378(HADHA)
NMEL: 110397114(HADHA)
ACYG: 106048383(HADHA)
LSR: 110478445(HADHA)
SCAN: 103824695(HADHA)
GFR: 102032411(HADHA)
FAB: 101815112(HADHA)
PHI: 102103703(HADHA)
PMAJ: 107198787(HADHA)
CCAE: 111927468(HADHA)
CCW: 104683133(HADHA)
ETL: 114071979(HADHA)
FPG: 101918262(HADHA)
FCH: 102045684(HADHA)
CLV: 102093055(HADHA)
EGZ: 104130955(HADHA)
NNI: 104014780(HADHA)
ACUN: 113478819(HADHA)
PADL: 103923913(HADHA)
AAM: 106499621(HADHA)
ASN: 102387874(HADHA)
AMJ: 102559143(HADHA)
PSS: 102461507(HADHA)
CMY: 102938765(HADHA)
CPIC: 101942783(HADHA)
ACS: 100553746(hadha)
PVT: 110090547(HADHA)
PBI: 103053277(HADHA)
PMUR: 107291031(HADHA) 107300505
TSR: 106541761(HADHA)
PMUA: 114594987(HADHA)
GJA: 107113769(HADHA)
XLA: 108717268(hadha.L) 444044(hadha.S)
XTR: 394832(hadha)
NPR: 108803249(HADHA)
DRE: 553401(hadhaa) 793834(hadhab)
IPU: 100304971 108257474(hadha)
TRU: 101073662(hadha)
LCO: 104929997(hadha)
NCC: 104942173(hadha)
OLA: 101155646(hadha)
XMA: 102229661(hadha)
XCO: 114133804(hadha)
PRET: 103458382(hadha)
CVG: 107095825(hadha)
NFU: 107392010(hadha)
KMR: 108239107(hadha)
ALIM: 106529789(hadha)
CSEM: 103380450(hadha)
POV: 109636387(hadha)
LCF: 108874899(hadha)
SDU: 111225708(hadha)
SLAL: 111673117(hadha)
MALB: 109964597(hadha)
SASA: 106603767 106611334(ECHA)
ELS: 105026863(hadha)
PKI: 111851531(hadha) 111857967
LCM: 102350823(HADHA)
CMK: 103183149(hadha)
RTP: 109910714(hadha)
BFO: 118404846
CIN: 100181980
SPU: 585357
APLC: 110978691
SKO: 100369576(HADHA)
DME: Dmel_CG4389(Mtpalpha)
DER: 6541344
DSE: 6611826
DSI: Dsimw501_GD22351(Dsim_GD22351)
DAN: 6504621
DSR: 110176800
DPE: 6589537
DMN: 108161786
DWI: 6641563
DAZ: 108610802
DNV: 108649592
DHE: 111603880
DVI: 6634031
MDE: 101889137
LCQ: 111679665
AAG: 5572923
AME: 410325
BIM: 100741897
BTER: 100642892
CCAL: 108623987
OBB: 114875921
SOC: 105201374
MPHA: 105837947
AEC: 105152057
ACEP: 105621618
PBAR: 105434016
VEM: 105567443
HST: 105189285
DQU: 106748121
CFO: 105254374
LHU: 105673815
PGC: 109863893
OBO: 105279823
PCF: 106784609
NVI: 100121740
CSOL: 105361087
MDL: 103579736
TCA: 662336
ATD: 109603223
NVL: 108558831
BMOR: 692820
BMAN: 114248364
PMAC: 106719824
PRAP: 110999633
HAW: 110371935
TNL: 113493488
PXY: 105387328
API: 100165657
DNX: 107168916
AGS: 114130103
RMD: 113555782
BTAB: 109036133
CLEC: 106670657
ZNE: 110827146
FCD: 110854334
PVM: 113805029
TUT: 107359547
DPTE: 113799485
CSCU: 111616495
CEL: CELE_C29F3.1(ech-1.1) CELE_T08B2.7(ech-1.2)
CBR: CBG12562
BMY: Bm1_51120
PCAN: 112574360
CRG: 105342066
OBI: 106883270
LAK: 106173898
EPA: 110254396
ADF: 107334024
AMIL: 114957985
PDAM: 113673361
SPIS: 111326946
DGT: 114535097
HMG: 100197152(hadha)
AQU: 100631983
FCY: FRACYDRAFT_183437(ECH_HADH2)
SPAR: SPRG_06557
 » show all
Reference
PMID:8135828
  Authors
Kamijo T, Aoyama T, Komiyama A, Hashimoto T
  Title
Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein.
  Journal
Biochem Biophys Res Commun 199:818-25 (1994)
DOI:10.1006/bbrc.1994.1302
  Sequence
[hsa:3030]

KEGG   ENZYME: 1.1.1.211
Entry
EC 1.1.1.211                Enzyme                                 

Name
long-chain-3-hydroxyacyl-CoA dehydrogenase;
beta-hydroxyacyl-CoA dehydrogenase;
long-chain 3-hydroxyacyl coenzyme A dehydrogenase;
3-hydroxyacyl-CoA dehydrogenase;
LCHAD
Class
Oxidoreductases;
Acting on the CH-OH group of donors;
With NAD+ or NADP+ as acceptor
Sysname
long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
Reaction(IUBMB)
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ = a long-chain 3-oxoacyl-CoA + NADH + H+ [RN:R01778]
Reaction(KEGG)
Substrate
long-chain (S)-3-hydroxyacyl-CoA;
NAD+ [CPD:C00003]
Product
long-chain 3-oxoacyl-CoA;
NADH [CPD:C00004];
H+ [CPD:C00080]
Comment
This enzyme was purified from the mitochondrial inner membrane. The enzyme has a preference for long-chain substrates, and activity with a C16 substrate was 6- to 15-fold higher than with a C4 substrate (cf. EC 1.1.1.35 3-hydroxyacyl-CoA dehydrogenase).
History
EC 1.1.1.211 created 1986
Pathway
ec00062  Fatty acid elongation
ec00071  Fatty acid degradation
ec01100  Metabolic pathways
Orthology
K07515  enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase
K10527  enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase
Genes
HSA: 3030(HADHA)
PTR: 459079(HADHA)
PPS: 100973698 100978217(HADHA)
GGO: 101143053(HADHA)
PON: 100172963(HADHA)
NLE: 100593214 100593233(HADHA)
MCC: 695975(HADHA)
MCF: 102142372(HADHA)
CSAB: 103220642(HADHA)
RRO: 104677892(HADHA)
RBB: 108532679(HADHA)
CJC: 100390439(HADHA)
SBQ: 101047074(HADHA)
MMU: 97212(Hadha)
MCAL: 110294690(Hadha)
MPAH: 110324206(Hadha)
RNO: 170670(Hadha)
MUN: 110547680(Hadha)
CGE: 100757947(Hadha)
NGI: 103735655(Hadha)
HGL: 101724137(Hadha)
CCAN: 109685831(Hadha)
OCU: 100355022(HADHA)
TUP: 102471941(HADHA) 102478660
CFA: 100856745(HADHA)
VVP: 112908532(HADHA)
AML: 100469654(HADHA)
UMR: 103671672(HADHA)
UAH: 113270772(HADHA)
ORO: 101364854(HADHA)
ELK: 111154088
FCA: 101084701(HADHA)
PTG: 102966470(HADHA)
PPAD: 109267284(HADHA)
AJU: 106967830(HADHA)
BTA: 281810(HADHA)
BOM: 102287426(HADHA)
BIU: 109566315(HADHA)
BBUB: 102398211(HADHA)
CHX: 102189046(HADHA)
OAS: 100192316(HADHA)
SSC: 397012(HADHA)
CFR: 102516648(HADHA)
CDK: 105098509(HADHA)
BACU: 103001885(HADHA)
LVE: 103086620(HADHA)
OOR: 101286957(HADHA)
DLE: 111174988(HADHA)
PCAD: 102996843(HADHA)
ECB: 100071381(HADHA)
EPZ: 103546462(HADHA)
EAI: 106840921(HADHA)
MYB: 102263028(HADHA)
MYD: 102751724(HADHA)
MNA: 107534200(HADHA)
HAI: 109383051(HADHA)
DRO: 112297764(HADHA)
PALE: 102889313(HADHA)
RAY: 107498535(HADHA)
MJV: 108392159(HADHA)
LAV: 100675857(HADHA)
TMU: 101353485
MDO: 100030776(HADHA)
SHR: 100928252(HADHA)
PCW: 110206510(HADHA)
OAA: 100077524(HADHA)
GGA: 395929(HADHA)
MGP: 100548080(HADHA)
CJO: 107312378(HADHA)
NMEL: 110397114(HADHA)
ACYG: 106048383(HADHA)
LSR: 110478445(HADHA)
SCAN: 103824695(HADHA)
GFR: 102032411(HADHA)
FAB: 101815112(HADHA)
PHI: 102103703(HADHA)
PMAJ: 107198787(HADHA)
CCAE: 111927468(HADHA)
CCW: 104683133(HADHA)
ETL: 114071979(HADHA)
FPG: 101918262(HADHA)
FCH: 102045684(HADHA)
CLV: 102093055(HADHA)
EGZ: 104130955(HADHA)
NNI: 104014780(HADHA)
ACUN: 113478819(HADHA)
PADL: 103923913(HADHA)
AAM: 106499621(HADHA)
ASN: 102387874(HADHA)
AMJ: 102559143(HADHA)
PSS: 102461507(HADHA)
CMY: 102938765(HADHA)
CPIC: 101942783(HADHA)
ACS: 100553746(hadha)
PVT: 110090547(HADHA)
PBI: 103053277(HADHA)
PMUR: 107291031(HADHA) 107300505
TSR: 106541761(HADHA)
PMUA: 114594987(HADHA)
GJA: 107113769(HADHA)
XLA: 108717268(hadha.L) 444044(hadha.S)
XTR: 394832(hadha)
NPR: 108803249(HADHA)
DRE: 553401(hadhaa) 793834(hadhab)
IPU: 100304971 108257474(hadha)
TRU: 101073662(hadha)
LCO: 104929997(hadha)
NCC: 104942173(hadha)
OLA: 101155646(hadha)
XMA: 102229661(hadha)
XCO: 114133804(hadha)
PRET: 103458382(hadha)
CVG: 107095825(hadha)
NFU: 107392010(hadha)
KMR: 108239107(hadha)
ALIM: 106529789(hadha)
CSEM: 103380450(hadha)
POV: 109636387(hadha)
LCF: 108874899(hadha)
SDU: 111225708(hadha)
SLAL: 111673117(hadha)
MALB: 109964597(hadha)
SASA: 106603767 106611334(ECHA)
ELS: 105026863(hadha)
PKI: 111851531(hadha) 111857967
LCM: 102350823(HADHA)
CMK: 103183149(hadha)
RTP: 109910714(hadha)
BFO: 118404846
CIN: 100181980
SPU: 585357
APLC: 110978691
SKO: 100369576(HADHA)
DME: Dmel_CG4389(Mtpalpha)
DER: 6541344
DSE: 6611826
DSI: Dsimw501_GD22351(Dsim_GD22351)
DAN: 6504621
DSR: 110176800
DPE: 6589537
DMN: 108161786
DWI: 6641563
DAZ: 108610802
DNV: 108649592
DHE: 111603880
DVI: 6634031
MDE: 101889137
LCQ: 111679665
AAG: 5572923
AME: 410325
BIM: 100741897
BTER: 100642892
CCAL: 108623987
OBB: 114875921
SOC: 105201374
MPHA: 105837947
AEC: 105152057
ACEP: 105621618
PBAR: 105434016
VEM: 105567443
HST: 105189285
DQU: 106748121
CFO: 105254374
LHU: 105673815
PGC: 109863893
OBO: 105279823
PCF: 106784609
NVI: 100121740
CSOL: 105361087
MDL: 103579736
TCA: 662336
ATD: 109603223
NVL: 108558831
BMOR: 692820
BMAN: 114248364
PMAC: 106719824
PRAP: 110999633
HAW: 110371935
TNL: 113493488
PXY: 105387328
API: 100165657
DNX: 107168916
AGS: 114130103
RMD: 113555782
BTAB: 109036133
CLEC: 106670657
ZNE: 110827146
FCD: 110854334
PVM: 113805029
TUT: 107359547
DPTE: 113799485
CSCU: 111616495
CEL: CELE_C29F3.1(ech-1.1) CELE_T08B2.7(ech-1.2)
CBR: CBG12562
BMY: Bm1_51120
PCAN: 112574360
CRG: 105342066
OBI: 106883270
LAK: 106173898
EPA: 110254396
ADF: 107334024
AMIL: 114957985
PDAM: 113673361
SPIS: 111326946
DGT: 114535097
HMG: 100197152(hadha)
AQU: 100631983
ATH: AT3G06860(MFP2) AT4G29010(AIM1)
CPAP: 110819879
LJA: Lj0g3v0340609.1(Lj0g3v0340609.1) Lj4g3v2310310.1(Lj4g3v2310310.1) Lj6g3v1465030.1(Lj6g3v1465030.1)
DOSA: Os01t0348600-01(Os01g0348600) Os02t0274100-01(Os02g0274100) Os05t0155000-01(Os05g0155000) Os05t0362100-01(Os05g0362100)
ATS: 109733511(LOC109733511) 109739968(LOC109739968) 109751016(LOC109751016) 109760663(LOC109760663) 109773312(LOC109773312)
APRO: F751_2626
FCY: FRACYDRAFT_183437(ECH_HADH2)
SPAR: SPRG_06557
 » show all
Reference
1  [PMID:7150615]
  Authors
El-Fakhri M, Middleton B.
  Title
The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria.
  Journal
Biochim Biophys Acta 713:270-9 (1982)
DOI:10.1016/0005-2760(82)90244-2
Other DBs
ExplorEnz - The Enzyme Database: 1.1.1.211
IUBMB Enzyme Nomenclature: 1.1.1.211
ExPASy - ENZYME nomenclature database: 1.1.1.211
BRENDA, the Enzyme Database: 1.1.1.211
CAS: 84177-52-6

KEGG   REACTION: R01778
Entry
R01778                      Reaction                               

Name
(3S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
Definition
(3S)-3-Hydroxyacyl-CoA + NAD+ <=> 3-Oxoacyl-CoA + NADH + H+
Equation
Comment
general reaction
the enzyme ec 1.1.1.35 has broad specificity to acyl chain-length, while ec 1.1.1.211 is long-chain specific
Reaction class
RC00001  C00003_C00004
RC00103  C00264_C00640
Enzyme
1.1.1.35        1.1.1.211
Pathway
rn00062  Fatty acid elongation
Module
M00085  Fatty acid elongation in mitochondria
Orthology
K00022  3-hydroxyacyl-CoA dehydrogenase [EC:1.1.1.35]
K07515  enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:4.2.1.17 1.1.1.211]
Other DBs
RHEA: 22435

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