KEGG   ENZYME: 4.1.1.94
Entry
EC 4.1.1.94                 Enzyme                                 
Name
ethylmalonyl-CoA decarboxylase
Class
Lyases;
Carbon-carbon lyases;
Carboxy-lyases
Sysname
(S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming)
Reaction(IUBMB)
(S)-ethylmalonyl-CoA = butanoyl-CoA + CO2 [RN:R09958]
Reaction(KEGG)
R09958
Substrate
(S)-ethylmalonyl-CoA [CPD:C18026]
Product
butanoyl-CoA [CPD:C00136];
CO2 [CPD:C00011]
Comment
The enzyme, which exists in all vertebrates, decarboxylates ethylmalonyl-CoA, a potentially toxic compound that is formed in low amounts by the activity of EC 6.4.1.2, acetyl-CoA carboxylase and EC 6.4.1.3, propanoyl-CoA carboxylase. It prefers the S isomer, and can decarboxylate (R)-ethylmalonyl-CoA with lower efficiency. cf. EC 7.2.4.1, (S)-methylmalonyl-CoA decarboxylase (sodium-transporting).
History
EC 4.1.1.94 created 2012
Orthology
K18426  ethylmalonyl-CoA/methylmalonyl-CoA decarboxylase
Genes
HSA55862(ECHDC1)
PTR745395(ECHDC1)
PPS100992035(ECHDC1)
GGO101134705(ECHDC1)
PON100173900(ECHDC1)
NLE100592701(ECHDC1)
HMH116471834(ECHDC1)
MCC712072(ECHDC1)
MCF102132641(ECHDC1)
MTHB126953270
MNI105465575(ECHDC1)
CSAB103240408(ECHDC1)
CATY105589458 105600484(ECHDC1)
PANU101016170(ECHDC1)
TGE112622317(ECHDC1)
MLEU105548860(ECHDC1)
RRO104665004(ECHDC1)
RBB108534272(ECHDC1)
TFN117097037(ECHDC1)
PTEH111554989(ECHDC1)
CANG105525884(ECHDC1)
CJC100400374(ECHDC1)
SBQ101054287(ECHDC1)
CIMI108307833(ECHDC1)
CSYR103268054(ECHDC1)
MMUR105878187(ECHDC1)
LCAT123633243(ECHDC1)
PCOQ105823603(ECHDC1)
OGA100949061(ECHDC1)
MMU52665(Echdc1)
MCAL110303168(Echdc1)
MPAH110338255(Echdc1)
RNO361465(Echdc1)
MCOC116097870(Echdc1)
ANU117701177(Echdc1)
MUN110557228(Echdc1)
CGE100764713(Echdc1)
MAUA101843111(Echdc1)
PROB127215093(Echdc1)
PLEU114692524(Echdc1)
MORG121449189(Echdc1)
MFOT126499119
AAMP119820353(Echdc1)
NGI103734575(Echdc1)
HGL101721458(Echdc1)
CPOC100723257 100728941(Echdc1)
CCAN109686373(Echdc1)
DORD105993597(Echdc1)
DSP122127034(Echdc1)
PLOP125357266(Echdc1)
NCAR124988747
MMMA107155200(Echdc1)
OCU100339488
OPI101520583(ECHDC1)
TUP102478961 102498422(ECHDC1)
GVR103583714(ECHDC1)
CFA609850(ECHDC1)
CLUD112644543(ECHDC1)
VVP112930904(ECHDC1)
VLG121477145 121485820(ECHDC1)
NPO129500438(ECHDC1)
AML100469062(ECHDC1)
UMR103663427(ECHDC1)
UAH113259426(ECHDC1)
UAR123797609(ECHDC1)
ELK111147650
LLV125103212
MPUF101680513(ECHDC1) 101693790
MNP132018145(ECHDC1)
MLK131833819(ECHDC1)
NVS122906857(ECHDC1)
ORO101378337(ECHDC1)
EJU114219464(ECHDC1)
ZCA113926576(ECHDC1)
MLX118000720(ECHDC1)
NSU110583662(ECHDC1)
LWW102726756(ECHDC1)
FCA101086310(ECHDC1)
PYU121030024(ECHDC1)
PCOO112862292(ECHDC1)
PBG122489964(ECHDC1)
PVIV125165318(ECHDC1)
LRUF124528307
PTG102958239(ECHDC1)
PPAD109248516(ECHDC1)
PUC125938903
AJU106976806
HHV120247810(ECHDC1)
BTA536284(ECHDC1)
BOM102265974(ECHDC1)
BIU109563748(ECHDC1)
BBUB102392783(ECHDC1)
BBIS105004216(ECHDC1)
CHX102178542(ECHDC1)
OAS101116321(ECHDC1)
BTAX128053325(ECHDC1)
ODA120862848(ECHDC1)
CCAD122422513(ECHDC1)
MBEZ129561337(ECHDC1)
SSC100512509(ECHDC1)
CFR102518806(ECHDC1)
CBAI105077204(ECHDC1)
CDK105096326(ECHDC1)
VPC102531989(ECHDC1)
BACU103017976(ECHDC1)
BMUS118905426(ECHDC1)
LVE103078139(ECHDC1)
OOR101270675(ECHDC1)
DLE111165431(ECHDC1)
PCAD102992135(ECHDC1)
PSIU116763214(ECHDC1)
NASI112409075(ECHDC1)
ECB100073045(ECHDC1)
EPZ103567873(ECHDC1)
EAI106838298(ECHDC1)
MYB102258272(ECHDC1)
MYD102751783(ECHDC1)
MMYO118660408(ECHDC1)
MLF102425047(ECHDC1)
PKL118705199(ECHDC1)
EFUS103283291(ECHDC1)
MNA107525980 107544237 107544476(ECHDC1)
DRO112297054(ECHDC1)
SHON118986719(ECHDC1)
AJM119044989(ECHDC1)
PDIC114495482(ECHDC1)
PHAS123821320(ECHDC1)
MMF118621864(ECHDC1)
PPAM129067762(ECHDC1)
HAI109392627(ECHDC1)
RFQ117020475(ECHDC1)
PALE102893310(ECHDC1)
PGIG120617338(ECHDC1)
PVP105291145(ECHDC1)
RAY107516901(ECHDC1)
TOD119252865(ECHDC1)
SETR126005854(ECHDC1)
LAV100657207(ECHDC1)
TMU101346877
ETF101653448(ECHDC1)
DNM101411251(ECHDC1)
MDO100030825(ECHDC1)
GAS123247882(ECHDC1)
SHR100919338(ECHDC1)
AFZ127562191
PCW110208850(ECHDC1)
OAA114809139(ECHDC1)
GGA769021(ECHDC1)
PCOC116240792(ECHDC1)
MGP100546546 104909791
CJO107311738(ECHDC1)
TPAI128078053(ECHDC1)
LMUT125689340(ECHDC1)
NMEL110395873(ECHDC1)
APLA101804132(ECHDC1)
ACYG106032483(ECHDC1)
CATA118243952(ECHDC1)
AFUL116487248(ECHDC1)
TGU100227322(ECHDC1)
LSR110479680(ECHDC1)
SCAN103818103(ECHDC1)
PMOA120501173(ECHDC1)
OTC121346147(ECHDC1)
PRUF121354802(ECHDC1)
GFR102044218(ECHDC1)
FAB101806230(ECHDC1)
OMA130251844(ECHDC1)
PHI102113663(ECHDC1)
PMAJ107201912(ECHDC1)
CCAE111927236(ECHDC1)
CCW104690802(ECHDC1)
CBRC103619418(ECHDC1)
ETL114055919(ECHDC1)
ZAB102061724(ECHDC1)
ZLE135445502(ECHDC1)
ACHL103810147(ECHDC1)
SVG106851908(ECHDC1)
MMEA130572133(ECHDC1)
HRT120749923(ECHDC1)
FPG101918396(ECHDC1)
FCH102055851(ECHDC1)
CLV102084911(ECHDC1)
EGZ104131447 104131979(ECHDC1)
NNI104008849(ECHDC1)
PCRI104029363(ECHDC1)
PLET104620274
EHS104508873(ECHDC1)
PCAO104041737(ECHDC1)
ACUN113478487(ECHDC1)
TALA104360780(ECHDC1)
PADL103918333(ECHDC1)
AFOR103900770(ECHDC1)
ACHC115349339(ECHDC1)
HALD104315673
HLE104828656(ECHDC1)
AGEN126046184
GCL127014757
CCRI104157724(ECHDC1)
CSTI104561419(ECHDC1)
CMAC104473158(ECHDC1)
MUI104538103(ECHDC1)
BREG104634838 104635680(ECHDC1)
FGA104081190(ECHDC1)
GSTE104256882(ECHDC1)
LDI104344787(ECHDC1)
MNB103776557(ECHDC1)
OHA104327043(ECHDC1)
NNT104403891(ECHDC1)
SHAB115609391(ECHDC1)
DPUB104308362(ECHDC1)
PGUU104463179(ECHDC1)
ACAR104523725(ECHDC1)
CPEA104390034(ECHDC1)
CVF104282079(ECHDC1)
RTD128907311(ECHDC1)
CUCA104061023(ECHDC1)
BRHI104495221(ECHDC1)
AAM106497960(ECHDC1)
AROW112968200(ECHDC1)
NPD112951379(ECHDC1)
TGT104566652
DNE112985989(ECHDC1)
SCAM104149296(ECHDC1)
ASN102383062(ECHDC1)
AMJ102560692(ECHDC1)
CPOO109312000(ECHDC1)
GGN109299794(ECHDC1)
PSS102451093(ECHDC1)
CMY102945116(ECHDC1)
CCAY125634193(ECHDC1)
DCC119853125(ECHDC1)
CPIC101939758(ECHDC1)
TST117874955(ECHDC1)
CABI116833326(ECHDC1)
MRV120400941(ECHDC1)
ACS100552293(echdc1)
ASAO132760953(ECHDC1)
PVT110077561(ECHDC1)
SUND121923063(ECHDC1)
PBI103055176(ECHDC1)
PMUR107287702(ECHDC1)
CTIG120299825(ECHDC1)
TSR106537842(ECHDC1)
PGUT117666251(ECHDC1)
APRI131192745(ECHDC1)
PTEX113439577(ECHDC1)
NSS113411165(ECHDC1)
VKO123020796(ECHDC1)
PMUA114594127(ECHDC1)
PRAF128410519(ECHDC1)
ZVI118081999(ECHDC1)
HCG128340517(ECHDC1)
GJA107121808(ECHDC1)
STOW125429768(ECHDC1)
EMC129331443(ECHDC1)
XLA496330(echdc1.L)
XTR496886(echdc1)
NPR108795294(ECHDC1)
RTEM120937299(ECHDC1)
BBUF120997063(ECHDC1)
BGAR122934623(ECHDC1)
MUO115465960(ECHDC1) 115465961
GSH117357122(ECHDC1)
DRE553585(echdc1)
SRX107726240
SANH107688988(echdc1)
SGH107569445 107580483
CCAR109098239(echdc1)
CAUA113060063(echdc1)
CGIB128031206
PTET122360180(echdc1)
LROH127179189(echdc1)
OMC131521416(echdc1)
PPRM120462653(echdc1)
RKG130090632(echdc1)
MAMB125243099(echdc1)
CIDE127497726
MASI127454419
TROS130549722(echdc1)
TDW130432351(echdc1)
MANU129449239(echdc1)
IPU108270580
IFU128614380(echdc1)
PHYP113526745(echdc1)
SMEO124384524(echdc1)
TFD113644222(echdc1)
TVC132845673(echdc1)
TRN134309888(echdc1)
AMEX103032922(echdc1)
CMAO118820087(echdc1)
EEE113572187(echdc1)
CHAR105908914(echdc1)
TRU101067683(echdc1)
TFS130532328(echdc1)
TNGGSTEN00035369G001
LCO104926210(echdc1)
NCC104955220(echdc1)
TBEN117480106(echdc1)
CGOB115021294(echdc1)
PGEO117460978(echdc1)
GACU117537438(echdc1)
EMAC134861964(echdc1)
ELY117265535(echdc1)
EFO125893204(echdc1)
PLEP121946058(echdc1)
SLUC116035226(echdc1)
ECRA117946797(echdc1)
ESP116690751(echdc1)
PFLV114556750(echdc1)
GAT120810187(echdc1)
PPUG119197660(echdc1)
AFB129109829(echdc1)
CLUM117745088(echdc1)
PSWI130191626(echdc1)
MSAM119897928(echdc1)
SCHU122881052(echdc1)
CUD121513091(echdc1)
ALAT119006856(echdc1)
MZE101482663(echdc1)
ONL100712005(echdc1)
OAU116322596(echdc1)
OLA101165254(echdc1)
OML112143315(echdc1)
CSAI133440919(echdc1)
XMA102231312(echdc1)
XCO114141955(echdc1)
XHE116717448(echdc1)
PRET103478333(echdc1)
PFOR103135856(echdc1)
PLAI106939841(echdc1)
PMEI106908798(echdc1)
GAF122831591(echdc1)
PPRL129367405(echdc1)
CVG107089614(echdc1)
CTUL119777051(echdc1)
GMU124863512(echdc1)
NFU107378790(echdc1)
KMR108232210(echdc1)
ALIM106536791(echdc1)
NWH119410190(echdc1)
AOCE111562493(echdc1)
MCEP125015767(echdc1)
CSEM103388602(echdc1)
POV109632074(echdc1)
SSEN122758626 122774405(echdc1)
HHIP117776391(echdc1)
HSP118114255(echdc1)
PPLT128449371(echdc1)
SMAU118314075(echdc1)
LCF108888597
SDU111227797(echdc1)
SLAL111658063(echdc1)
XGL120783598(echdc1)
HCQ109522145 109525601 109531047
SSCV125975482
SBIA133500385(echdc1)
PEE133402011(echdc1)
PTAO133479150(echdc1)
BPEC110171745(echdc1)
MALB109958274(echdc1)
BSPL114843697(echdc1)
SJO128366373(echdc1)
SASA106572885 106599002
STRU115187901 115187916
OTW112239354 112239356
OMY110510292(echdc1)
OGO124017640 124017641
ONE115127936(echdc1)
OKI109885705 116359282(echdc1)
OKE127923452(echdc1)
SALP112077607(echdc1)
SNH120040890(echdc1)
CCLU121542579 121585430
ELS105023514(echdc1)
SFM108937328(echdc1)
PKI111844541(echdc1)
AANG118214601(echdc1)
LOC102682286(echdc1)
PSPA121316267(echdc1)
ARUT117402543(echdc1)
PSEX120525485(echdc1)
LCM102352882(ECHDC1)
CMK103182298(echdc1)
RTP109917264(echdc1)
CPLA122544525(echdc1)
HOC132806127(echdc1)
LERI129697272(echdc1)
PMRN116946686(ECHDC1)
LRJ133353012(ECHDC1)
BFO118410251
BBEL109480902
SCLV120339755 120339995
SPU115919003 584418
LPIC129280803
APLC110980172
AJC117124092
TCA659052
SOY115880460
AGB108914754
DVV126887417
NVL108560577
APLN108739264 112904572
PPYR116161769 116162538
API100573761
DNX107166647
AGS114128566 114128574
RMD113558438 113558439
ACOO126844587
DVT126899424
BTAB109038214
CLEC106664392
HHAL106688853
HVI124368957
MQU128983706
PHUPhum_PHUM171680
FOC113212778
TPAL117654386
ZNE110830973
CSEC111866308
SGRE126278196
BROR134539235
FCD110851854
DPXDAPPUDRAFT_335707
DPZ124311305
PVM113812256
PJA122246691
PCHN125037073
HAME121872606
PCLA123759394
CQD128704785
PTRU123505401
ESN126987114
MNZ135200316
HAZT108680841
EAF111707020
LSM121125969
PPOI119099484
DSV119430995 119431281
RSAN119371844 119371847
RMP119163844 119163845
TUT107363239
DPTE113799364
DFR124496270
CSCU111639305
LPOL106472084
PMEO129595958
CELCELE_C32E8.9(C32E8.9)
CBRCBG_12007
CRQGCK72_002184
NAINECAME_01713
HROHELRODRAFT_159708 HELRODRAFT_159709
LGILOTGIDRAFT_116748
PVUL126818663
PCAN112573749
BGT106062219 106064380
GAE121375161
HRF124112771
HRJ124265295
CRG105325046
CANU128164236
MYI110456022
PMAX117325253
MCAF127736928
MMER123525273
RPHI132748831
DPOL127853596
OBI106867393
OSN115214032
LLON135486321 135487116
NVE5507440
EPA110242745
ATEN116294421
ADF107335744
AMIL114950263
PDAM113672137
SPIS111335182
DGT114530270
XEN124438799
HMG100209361
TADTRIADDRAFT_53519
MBRMONBRDRAFT_10298
SREPTSG_02237
PIFPITG_03865
PSOJPHYSODRAFT_349249 PHYSODRAFT_466815
SPARSPRG_12932
 » show all
Reference
1  [PMID:22016388]
  Authors
Linster CL, Noel G, Stroobant V, Vertommen D, Vincent MF, Bommer GT, Veiga-da-Cunha M, Van Schaftingen E
  Title
Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading.
  Journal
J Biol Chem 286:42992-3003 (2011)
DOI:10.1074/jbc.M111.281527
  Sequence
[hsa:55862] [mmu:52665] [rno:361465]
Other DBs
ExplorEnz - The Enzyme Database: 4.1.1.94
IUBMB Enzyme Nomenclature: 4.1.1.94
ExPASy - ENZYME nomenclature database: 4.1.1.94
BRENDA, the Enzyme Database: 4.1.1.94

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