KEGG ENZYME: 4.1.1.94

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

HSA:	55862(ECHDC1)

PTR:	745395(ECHDC1)

PPS:	100992035(ECHDC1)

GGO:	101134705(ECHDC1)

PON:	100173900(ECHDC1)

NLE:	100592701(ECHDC1)

HMH:	116471834(ECHDC1)

MCC:	712072(ECHDC1)

MCF:	102132641(ECHDC1)

MTHB:

126953270

MNI:	105465575(ECHDC1)

CSAB:

103240408(ECHDC1)

CATY:

105589458 105600484(ECHDC1)

PANU:

101016170(ECHDC1)

TGE:	112622317(ECHDC1)

MLEU:

105548860(ECHDC1)

RRO:	104665004(ECHDC1)

RBB:	108534272(ECHDC1)

TFN:	117097037(ECHDC1)

PTEH:

111554989(ECHDC1)

CANG:

105525884(ECHDC1)

CJC:	100400374(ECHDC1)

SBQ:	101054287(ECHDC1)

CIMI:

108307833(ECHDC1)

CSYR:

103268054(ECHDC1)

MMUR:

105878187(ECHDC1)

LCAT:

123633243(ECHDC1)

PCOQ:

105823603(ECHDC1)

OGA:	100949061(ECHDC1)

MMU:	52665(Echdc1)

MCAL:

110303168(Echdc1)

MPAH:

110338255(Echdc1)

RNO:	361465(Echdc1)

MCOC:

116097870(Echdc1)

ANU:	117701177(Echdc1)

MUN:	110557228(Echdc1)

CGE:	100764713(Echdc1)

MAUA:

101843111(Echdc1)

PROB:

127215093(Echdc1)

PLEU:

114692524(Echdc1)

MORG:

121449189(Echdc1)

MFOT:

126499119

AAMP:

119820353(Echdc1)

NGI:	103734575(Echdc1)

HGL:	101721458(Echdc1)

CPOC:

100723257 100728941(Echdc1)

CCAN:

109686373(Echdc1)

DORD:

105993597(Echdc1)

DSP:	122127034(Echdc1)

PLOP:

125357266(Echdc1)

NCAR:

124988747

MMMA:

107155200(Echdc1)

OCU:

100339488

OPI:	101520583(ECHDC1)

TUP:	102478961 102498422(ECHDC1)

GVR:	103583714(ECHDC1)

CFA:	609850(ECHDC1)

CLUD:

112644543(ECHDC1)

VVP:	112930904(ECHDC1)

VLG:	121477145 121485820(ECHDC1)

NPO:	129500438(ECHDC1)

AML:	100469062(ECHDC1)

UMR:	103663427(ECHDC1)

UAH:	113259426(ECHDC1)

UAR:	123797609(ECHDC1)

ELK:

111147650

LLV:

125103212

MPUF:

101680513(ECHDC1) 101693790

MNP:	132018145(ECHDC1)

MLK:	131833819(ECHDC1)

NVS:	122906857(ECHDC1)

ORO:	101378337(ECHDC1)

EJU:	114219464(ECHDC1)

ZCA:	113926576(ECHDC1)

MLX:	118000720(ECHDC1)

NSU:	110583662(ECHDC1)

LWW:	102726756(ECHDC1)

FCA:	101086310(ECHDC1)

PYU:	121030024(ECHDC1)

PCOO:

112862292(ECHDC1)

PBG:	122489964(ECHDC1)

PVIV:

125165318(ECHDC1)

LRUF:

124528307

PTG:	102958239(ECHDC1)

PPAD:

109248516(ECHDC1)

PUC:

125938903

AJU:

106976806

HHV:	120247810(ECHDC1)

BTA:	536284(ECHDC1)

BOM:	102265974(ECHDC1)

BIU:	109563748(ECHDC1)

BBUB:

102392783(ECHDC1)

BBIS:

105004216(ECHDC1)

CHX:	102178542(ECHDC1)

OAS:	101116321(ECHDC1)

BTAX:

128053325(ECHDC1)

ODA:	120862848(ECHDC1)

CCAD:

122422513(ECHDC1)

MBEZ:

129561337(ECHDC1)

SSC:	100512509(ECHDC1)

CFR:	102518806(ECHDC1)

CBAI:

105077204(ECHDC1)

CDK:	105096326(ECHDC1)

VPC:	102531989(ECHDC1)

BACU:

103017976(ECHDC1)

BMUS:

118905426(ECHDC1)

LVE:	103078139(ECHDC1)

OOR:	101270675(ECHDC1)

DLE:	111165431(ECHDC1)

PCAD:

102992135(ECHDC1)

PSIU:

116763214(ECHDC1)

NASI:

112409075(ECHDC1)

ECB:	100073045(ECHDC1)

EPZ:	103567873(ECHDC1)

EAI:	106838298(ECHDC1)

MYB:	102258272(ECHDC1)

MYD:	102751783(ECHDC1)

MMYO:

118660408(ECHDC1)

MLF:	102425047(ECHDC1)

PKL:	118705199(ECHDC1)

EFUS:

103283291(ECHDC1)

MNA:	107525980 107544237 107544476(ECHDC1)

DRO:	112297054(ECHDC1)

SHON:

118986719(ECHDC1)

AJM:	119044989(ECHDC1)

PDIC:

114495482(ECHDC1)

PHAS:

123821320(ECHDC1)

MMF:	118621864(ECHDC1)

PPAM:

129067762(ECHDC1)

HAI:	109392627(ECHDC1)

RFQ:	117020475(ECHDC1)

PALE:

102893310(ECHDC1)

PGIG:

120617338(ECHDC1)

PVP:	105291145(ECHDC1)

RAY:	107516901(ECHDC1)

TOD:	119252865(ECHDC1)

LAV:	100657207(ECHDC1)

TMU:

101346877

ETF:	101653448(ECHDC1)

DNM:	101411251(ECHDC1)

MDO:	100030825(ECHDC1)

GAS:	123247882(ECHDC1)

SHR:	100919338(ECHDC1)

AFZ:

127562191

PCW:	110208850(ECHDC1)

OAA:	114809139(ECHDC1)

GGA:	769021(ECHDC1)

PCOC:

116240792(ECHDC1)

MGP:	100546546 104909791

CJO:	107311738(ECHDC1)

TPAI:

128078053(ECHDC1)

LMUT:

125689340(ECHDC1)

NMEL:

110395873(ECHDC1)

APLA:

101804132(ECHDC1)

ACYG:

106032483(ECHDC1)

CATA:

118243952(ECHDC1)

AFUL:

116487248(ECHDC1)

TGU:	100227322(ECHDC1)

LSR:	110479680(ECHDC1)

SCAN:

103818103(ECHDC1)

PMOA:

120501173(ECHDC1)

OTC:	121346147(ECHDC1)

PRUF:

121354802(ECHDC1)

GFR:	102044218(ECHDC1)

FAB:	101806230(ECHDC1)

OMA:	130251844(ECHDC1)

PHI:	102113663(ECHDC1)

PMAJ:

107201912(ECHDC1)

CCAE:

111927236(ECHDC1)

CCW:	104690802(ECHDC1)

CBRC:

103619418(ECHDC1)

ETL:	114055919(ECHDC1)

ZAB:	102061724(ECHDC1)

ACHL:

103810147(ECHDC1)

SVG:	106851908(ECHDC1)

MMEA:

130572133(ECHDC1)

HRT:	120749923(ECHDC1)

FPG:	101918396(ECHDC1)

FCH:	102055851(ECHDC1)

CLV:	102084911(ECHDC1)

EGZ:	104131447 104131979(ECHDC1)

NNI:	104008849(ECHDC1)

PCRI:

104029363(ECHDC1)

PLET:

104620274

EHS:	104508873(ECHDC1)

PCAO:

104041737(ECHDC1)

ACUN:

113478487(ECHDC1)

TALA:

104360780(ECHDC1)

PADL:

103918333(ECHDC1)

AFOR:

103900770(ECHDC1)

ACHC:

115349339(ECHDC1)

HALD:

104315673

HLE:	104828656(ECHDC1)

AGEN:

126046184

GCL:

127014757

CCRI:

104157724(ECHDC1)

CSTI:

104561419(ECHDC1)

CMAC:

104473158(ECHDC1)

MUI:	104538103(ECHDC1)

BREG:

104634838 104635680(ECHDC1)

FGA:	104081190(ECHDC1)

GSTE:

104256882(ECHDC1)

LDI:	104344787(ECHDC1)

MNB:	103776557(ECHDC1)

OHA:	104327043(ECHDC1)

NNT:	104403891(ECHDC1)

SHAB:

115609391(ECHDC1)

DPUB:

104308362(ECHDC1)

PGUU:

104463179(ECHDC1)

ACAR:

104523725(ECHDC1)

CPEA:

104390034(ECHDC1)

CVF:	104282079(ECHDC1)

RTD:	128907311(ECHDC1)

CUCA:

104061023(ECHDC1)

BRHI:

104495221(ECHDC1)

AAM:	106497960(ECHDC1)

AROW:

112968200(ECHDC1)

NPD:	112951379(ECHDC1)

TGT:

104566652

DNE:	112985989(ECHDC1)

SCAM:

104149296(ECHDC1)

ASN:	102383062(ECHDC1)

AMJ:	102560692(ECHDC1)

CPOO:

109312000(ECHDC1)

GGN:	109299794(ECHDC1)

PSS:	102451093(ECHDC1)

CMY:	102945116(ECHDC1)

CCAY:

125634193(ECHDC1)

DCC:	119853125(ECHDC1)

CPIC:

101939758(ECHDC1)

TST:	117874955(ECHDC1)

CABI:

116833326(ECHDC1)

MRV:	120400941(ECHDC1)

ACS:	100552293(echdc1)

ASAO:

132760953(ECHDC1)

PVT:	110077561(ECHDC1)

SUND:

121923063(ECHDC1)

PBI:	103055176(ECHDC1)

PMUR:

107287702(ECHDC1)

CTIG:

120299825(ECHDC1)

TSR:	106537842(ECHDC1)

PGUT:

117666251(ECHDC1)

APRI:

131192745(ECHDC1)

PTEX:

113439577(ECHDC1)

NSS:	113411165(ECHDC1)

VKO:	123020796(ECHDC1)

PMUA:

114594127(ECHDC1)

PRAF:

128410519(ECHDC1)

ZVI:	118081999(ECHDC1)

HCG:	128340517(ECHDC1)

GJA:	107121808(ECHDC1)

STOW:

125429768(ECHDC1)

EMC:	129331443(ECHDC1)

XLA:	496330(echdc1.L)

XTR:	496886(echdc1)

NPR:	108795294(ECHDC1)

RTEM:

120937299(ECHDC1)

BBUF:

120997063(ECHDC1)

BGAR:

122934623(ECHDC1)

MUO:	115465960(ECHDC1) 115465961

GSH:	117357122(ECHDC1)

DRE:	553585(echdc1)

SRX:

107726240

SANH:

107688988(echdc1)

SGH:	107569445 107580483

CCAR:

109098239(echdc1)

CAUA:

113060063(echdc1)

CGIB:

128031206

PTET:

122360180(echdc1)

LROH:

127179189(echdc1)

OMC:	131521416(echdc1)

PPRM:

120462653(echdc1)

RKG:	130090632(echdc1)

MAMB:

125243099(echdc1)

CIDE:

127497726

MASI:

127454419

TROS:

130549722(echdc1)

TDW:	130432351(echdc1)

MANU:

129449239(echdc1)

IPU:

108270580

IFU:	128614380(echdc1)

PHYP:

113526745(echdc1)

SMEO:

124384524(echdc1)

TFD:	113644222(echdc1)

TVC:	132845673(echdc1)

AMEX:

103032922(echdc1)

CMAO:

118820087(echdc1)

EEE:	113572187(echdc1)

CHAR:

105908914(echdc1)

TRU:	101067683(echdc1)

TFS:	130532328(echdc1)

TNG:	GSTEN00035369G001

LCO:	104926210(echdc1)

NCC:	104955220(echdc1)

TBEN:

117480106(echdc1)

CGOB:

115021294(echdc1)

PGEO:

117460978(echdc1)

GACU:

117537438(echdc1)

EMAC:

134861964(echdc1)

ELY:	117265535(echdc1)

EFO:	125893204(echdc1)

PLEP:

121946058(echdc1)

SLUC:

116035226(echdc1)

ECRA:

117946797(echdc1)

ESP:	116690751(echdc1)

PFLV:

114556750(echdc1)

GAT:	120810187(echdc1)

PPUG:

119197660(echdc1)

AFB:	129109829(echdc1)

CLUM:

117745088(echdc1)

PSWI:

130191626(echdc1)

MSAM:

119897928(echdc1)

SCHU:

122881052(echdc1)

CUD:	121513091(echdc1)

ALAT:

119006856(echdc1)

MZE:	101482663(echdc1)

ONL:	100712005(echdc1)

OAU:	116322596(echdc1)

OLA:	101165254(echdc1)

OML:	112143315(echdc1)

CSAI:

133440919(echdc1)

XMA:	102231312(echdc1)

XCO:	114141955(echdc1)

XHE:	116717448(echdc1)

PRET:

103478333(echdc1)

PFOR:

103135856(echdc1)

PLAI:

106939841(echdc1)

PMEI:

106908798(echdc1)

GAF:	122831591(echdc1)

PPRL:

129367405(echdc1)

CVG:	107089614(echdc1)

CTUL:

119777051(echdc1)

GMU:	124863512(echdc1)

NFU:	107378790(echdc1)

KMR:	108232210(echdc1)

ALIM:

106536791(echdc1)

NWH:	119410190(echdc1)

AOCE:

111562493(echdc1)

MCEP:

125015767(echdc1)

CSEM:

103388602(echdc1)

POV:	109632074(echdc1)

SSEN:

122758626 122774405(echdc1)

HHIP:

117776391(echdc1)

HSP:	118114255(echdc1)

SMAU:

118314075(echdc1)

LCF:

108888597

SDU:	111227797(echdc1)

SLAL:

111658063(echdc1)

XGL:	120783598(echdc1)

HCQ:	109522145 109525601 109531047

SSCV:

125975482

SBIA:

133500385(echdc1)

PEE:	133402011(echdc1)

PTAO:

133479150(echdc1)

BPEC:

110171745(echdc1)

MALB:

109958274(echdc1)

BSPL:

114843697(echdc1)

SJO:	128366373(echdc1)

SASA:

106572885 106599002

STRU:

115187901 115187916

OTW:	112239354 112239356

OMY:	110510292(echdc1)

OGO:	124017640 124017641

ONE:	115127936(echdc1)

OKI:	109885705 116359282(echdc1)

OKE:	127923452(echdc1)

SALP:

112077607(echdc1)

SNH:	120040890(echdc1)

CCLU:

121542579 121585430

ELS:	105023514(echdc1)

SFM:	108937328(echdc1)

PKI:	111844541(echdc1)

AANG:

118214601(echdc1)

LOC:	102682286(echdc1)

PSPA:

121316267(echdc1)

ARUT:

117402543(echdc1)

PSEX:

120525485(echdc1)

LCM:	102352882(ECHDC1)

CMK:	103182298(echdc1)

RTP:	109917264(echdc1)

CPLA:

122544525(echdc1)

HOC:	132806127(echdc1)

LERI:

129697272(echdc1)

PMRN:

116946686(ECHDC1)

LRJ:	133353012(ECHDC1)

BFO:

118410251

BBEL:

109480902

SCLV:

120339755 120339995

SPU:	115919003 584418

LPIC:

129280803

APLC:

110980172

AJC:

117124092

TCA:

659052

SOY:

115880460

AGB:

108914754

NVL:

108560577

APLN:

108739264 112904572

PPYR:

116161769 116162538

API:

100573761

DNX:

107166647

AGS:	114128566 114128574

RMD:	113558438 113558439

ACOO:

126844587

DVT:

126899424

BTAB:

109038214

CLEC:

106664392

HHAL:

106688853

HVI:

124368957

MQU:

128983706

PHU:	Phum_PHUM171680

FOC:

113212778

TPAL:

117654386

ZNE:

110830973

CSEC:

111866308

SGRE:

126278196

BROR:

134539235

FCD:

110851854

DPX:	DAPPUDRAFT_335707

DPZ:

124311305

PVM:

113812256

PJA:

122246691

PCHN:

125037073

HAME:

121872606

PCLA:

123759394

CQD:

128704785

PTRU:

123505401

ESN:

126987114

HAZT:

108680841

EAF:

111707020

LSM:

121125969

PPOI:

119099484

DSV:	119430995 119431281

RSAN:

119371844 119371847

RMP:	119163844 119163845

TUT:

107363239

DPTE:

113799364

DFR:

124496270

CSCU:

111639305

LPOL:

106472084

PMEO:

129595958

CEL:	CELE_C32E8.9(C32E8.9)

CBR:

CBG_12007

CRQ:	GCK72_002184

NAI:	NECAME_01713

HRO:	HELRODRAFT_159708 HELRODRAFT_159709

LGI:	LOTGIDRAFT_116748

PVUL:

126818663

PCAN:

112573749

BGT:	106062219 106064380

GAE:

121375161

HRF:

124112771

HRJ:

124265295

CRG:

105325046

CANU:

128164236

MYI:

110456022

PMAX:

117325253

MCAF:

127736928

MMER:

123525273

RPHI:

132748831

DPOL:

127853596

OBI:

106867393

OSN:

115214032

NVE:

5507440

EPA:

110242745

ATEN:

116294421

ADF:

107335744

AMIL:

114950263

PDAM:

113672137

SPIS:

111335182

DGT:

114530270

XEN:

124438799

HMG:

100209361

TAD:	TRIADDRAFT_53519

MBR:	MONBRDRAFT_10298

SRE:	PTSG_02237

PIF:	PITG_03865

PSOJ:

PHYSODRAFT_349249 PHYSODRAFT_466815

SPAR:

SPRG_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

DBGET integrated database retrieval system