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
HSA: 55862(ECHDC1)
PTR: 745395(ECHDC1)
PPS: 100992035(ECHDC1)
GGO: 101134705(ECHDC1)
PON: 100173900(ECHDC1)
NLE: 100592701(ECHDC1)
MCC: 712072(ECHDC1)
MCF: 102132641(ECHDC1)
CSAB: 103240408(ECHDC1)
CATY: 105589458 105600484(ECHDC1)
PANU: 101016170(ECHDC1)
RRO: 104665004(ECHDC1)
RBB: 108534272(ECHDC1)
TFN: 117097037(ECHDC1)
PTEH: 111554989(ECHDC1)
CJC: 100400374(ECHDC1)
SBQ: 101054287(ECHDC1)
MMUR: 105878187(ECHDC1)
MMU: 52665(Echdc1)
MCAL: 110303168(Echdc1)
MPAH: 110338255(Echdc1)
RNO: 361465(Echdc1)
MCOC: 116097870(Echdc1)
MUN: 110557228(Echdc1)
CGE: 100764713(Echdc1)
PLEU: 114692524(Echdc1)
NGI: 103734575(Echdc1)
HGL: 101721458(Echdc1)
CCAN: 109686373(Echdc1)
OCU: 100339488(ECHDC1)
TUP: 102478961 102498422(ECHDC1)
CFA: 609850(ECHDC1)
VVP: 112930904(ECHDC1)
VLG: 121477145 121485820(ECHDC1)
AML: 100469062(ECHDC1)
UMR: 103663427(ECHDC1)
UAH: 113259426(ECHDC1)
ORO: 101378337(ECHDC1)
ELK: 111147650
MPUF: 101680513(ECHDC1) 101693790
EJU: 114219464(ECHDC1)
MLX: 118000720(ECHDC1)
FCA: 101086310(ECHDC1)
PTG: 102958239(ECHDC1)
PPAD: 109248516(ECHDC1)
AJU: 106976806(ECHDC1)
HHV: 120247810(ECHDC1)
BTA: 536284(ECHDC1)
BOM: 102265974(ECHDC1)
BIU: 109563748(ECHDC1)
BBUB: 102392783(ECHDC1)
CHX: 102178542(ECHDC1)
OAS: 101116321(ECHDC1)
ODA: 120862848(ECHDC1)
SSC: 100512509(ECHDC1)
CFR: 102518806(ECHDC1)
CBAI: 105077204(ECHDC1)
CDK: 105096326(ECHDC1)
BACU: 103017976(ECHDC1)
LVE: 103078139(ECHDC1)
OOR: 101270675(ECHDC1)
DLE: 111165431(ECHDC1)
PCAD: 102992135(ECHDC1)
ECB: 100073045(ECHDC1)
EPZ: 103567873(ECHDC1)
EAI: 106838298 106838937(ECHDC1)
MYB: 102258272(ECHDC1)
MYD: 102751783(ECHDC1)
MMYO: 118660408(ECHDC1)
HAI: 109392627(ECHDC1)
DRO: 112297054(ECHDC1)
SHON: 118986719(ECHDC1)
AJM: 119044989(ECHDC1)
MMF: 118621864(ECHDC1)
PALE: 102893310(ECHDC1)
PGIG: 120617338(ECHDC1)
RAY: 107516901(ECHDC1)
MJV: 108394179(ECHDC1)
TOD: 119252865(ECHDC1)
LAV: 100657207(ECHDC1)
TMU: 101346877
MDO: 100030825(ECHDC1)
SHR: 100919338(ECHDC1)
PCW: 110208850(ECHDC1)
OAA: 114809139(ECHDC1)
GGA: 769021(ECHDC1)
PCOC: 116240792(ECHDC1)
CJO: 107311738(ECHDC1)
NMEL: 110395873(ECHDC1)
APLA: 101804132(ECHDC1)
ACYG: 106032483(ECHDC1)
TGU: 100227322(ECHDC1)
LSR: 110479680(ECHDC1)
SCAN: 103818103(ECHDC1)
PMOA: 120501173(ECHDC1)
GFR: 102044218(ECHDC1)
FAB: 101806230(ECHDC1)
PHI: 102113663(ECHDC1)
PMAJ: 107201912(ECHDC1)
CCAE: 111927236(ECHDC1)
CCW: 104690802(ECHDC1)
ETL: 114055919(ECHDC1)
FPG: 101918396(ECHDC1)
FCH: 102055851(ECHDC1)
CLV: 102084911(ECHDC1)
EGZ: 104131447 104131979(ECHDC1)
NNI: 104008849(ECHDC1)
ACUN: 113478487(ECHDC1)
PADL: 103918333(ECHDC1)
AAM: 106497960(ECHDC1)
ASN: 102383062(ECHDC1)
AMJ: 102560692(ECHDC1)
CPOO: 109312000(ECHDC1)
GGN: 109299794(ECHDC1)
PSS: 102451093(ECHDC1)
CMY: 102945116(ECHDC1)
CPIC: 101939758(ECHDC1)
TST: 117874955(ECHDC1)
CABI: 116833326(ECHDC1)
ACS: 100552293(echdc1)
PVT: 110077561(ECHDC1)
PBI: 103055176(ECHDC1)
PMUR: 107287702(ECHDC1)
TSR: 106537842(ECHDC1)
PGUT: 117666251(ECHDC1)
PMUA: 114594127(ECHDC1)
ZVI: 118081999(ECHDC1)
GJA: 107121808(ECHDC1)
XLA: 496330(echdc1.L)
XTR: 496886(echdc1)
NPR: 108795294(ECHDC1)
DRE: 553585(echdc1)
SRX: 107726240
SANH: 107688988(echdc1)
CCAR: 109098239(echdc1)
CAUA: 113060063(echdc1)
IPU: 108270580(echdc1)
PHYP: 113526745(echdc1)
AMEX: 103032922(echdc1)
EEE: 113572187(echdc1)
TRU: 101067683(echdc1)
LCO: 104926210(echdc1)
NCC: 104955220(echdc1)
CGOB: 115021294(echdc1)
ELY: 117265535(echdc1)
PLEP: 121946058(echdc1)
SLUC: 116035226(echdc1)
ECRA: 117946797(echdc1)
PFLV: 114556750(echdc1)
GAT: 120810187(echdc1)
MSAM: 119897928(echdc1)
CUD: 121513091(echdc1)
MZE: 101482663(echdc1)
ONL: 100712005(echdc1)
OAU: 116322596(echdc1)
OLA: 101165254(echdc1)
OML: 112143315(echdc1)
XMA: 102231312(echdc1)
XCO: 114141955(echdc1)
XHE: 116717448(echdc1)
PRET: 103478333(echdc1)
CVG: 107089614(echdc1)
CTUL: 119777051(echdc1)
NFU: 107378790(echdc1)
KMR: 108232210(echdc1)
ALIM: 106536791(echdc1)
AOCE: 111562493(echdc1)
CSEM: 103388602(echdc1)
POV: 109632074(echdc1)
LCF: 108888597(echdc1)
SDU: 111227797(echdc1)
SLAL: 111658063(echdc1)
XGL: 120783598(echdc1)
BPEC: 110171745(echdc1)
MALB: 109958274(echdc1)
OMY: 110510292(echdc1)
SALP: 112077607(echdc1)
SNH: 120040890(echdc1)
ELS: 105023514(echdc1)
SFM: 108937328(echdc1)
PKI: 111844541(echdc1)
AANG: 118214601(echdc1)
LOC: 102682286(echdc1)
PSPA: 121316267(echdc1)
ARUT: 117402543(echdc1)
LCM: 102352882(ECHDC1)
CMK: 103182298(echdc1)
RTP: 109917264(echdc1)
BFO: 118410251
BBEL: 109480902
APLC: 110980172
TCA: 659052
ATD: 109594706
AGB: 108914754
NVL: 108560577
API: 100573761
DNX: 107166647
BTAB: 109038214
CLEC: 106664392
HHAL: 106688853
ZNE: 110830973
CSEC: 111866308
FCD: 110851854
PVM: 113812256
HAME: 121872606
HAZT: 108680841
EAF: 111707020
TUT: 107363239
DPTE: 113799364
CSCU: 111639305
CEL: CELE_C32E8.9(C32E8.9)
CBR: CBG12007
PCAN: 112573749
GAE: 121375161
CRG: 105325046
MYI: 110456022
PMAX: 117325253
OBI: 106867393
NVE: 5507440
EPA: 110242745
ATEN: 116294421
ADF: 107335744
AMIL: 114950263
PDAM: 113672137
SPIS: 111335182
DGT: 114530270
HMG: 100209361
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

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