KEGG   ENZYME: 4.1.1.94Help
Entry
EC 4.1.1.94                 Enzyme                                 

Name
ethylmalonyl-CoA decarboxylase
Class
Lyases;
Carbon-carbon lyases;
Carboxy-lyases
BRITE hierarchy
Sysname
(S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming)
Reaction(IUBMB)
(S)-ethylmalonyl-CoA = butanoyl-CoA + CO2 [RN:R09958]
Reaction(KEGG)
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)-methylmalonyl-CoA with lower efficiency. cf. EC 4.1.1.41 (methylmalonyl-CoA decarboxylase).
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)
RRO: 104665004(ECHDC1)
RBB: 108534272(ECHDC1)
CJC: 100400374(ECHDC1)
SBQ: 101054287(ECHDC1)
MMU: 52665(Echdc1)
RNO: 361465(Echdc1)
CGE: 100764713(Echdc1)
NGI: 103734575(Echdc1)
HGL: 101721458(Echdc1)
CCAN: 109686373(Echdc1)
OCU: 100339488(ECHDC1)
TUP: 102478961 102498422(ECHDC1)
CFA: 609850(ECHDC1)
AML: 100469062(ECHDC1)
UMR: 103663427(ECHDC1)
ORO: 101378337(ECHDC1)
FCA: 101086310(ECHDC1)
PTG: 102958239(ECHDC1)
AJU: 106976806(ECHDC1)
BTA: 536284(ECHDC1)
BOM: 102265974(ECHDC1)
BIU: 109563748(ECHDC1)
PHD: 102336638(ECHDC1)
CHX: 102178542(ECHDC1)
OAS: 101116321(ECHDC1)
SSC: 100512509(ECHDC1)
CFR: 102518806(ECHDC1)
CDK: 105096326(ECHDC1)
BACU: 103017976(ECHDC1)
LVE: 103078139(ECHDC1)
OOR: 101270675(ECHDC1)
ECB: 100073045(ECHDC1)
EPZ: 103567873(ECHDC1)
EAI: 106838298 106838937(ECHDC1)
MYB: 102258272(ECHDC1)
MYD: 102751783(ECHDC1)
HAI: 109392627(ECHDC1)
RSS: 109446256(ECHDC1)
PALE: 102893310(ECHDC1)
LAV: 100657207(ECHDC1)
TMU: 101346877
MDO: 100030825(ECHDC1)
SHR: 100919338(ECHDC1)
GGA: 769021(ECHDC1)
MGP: 100546546
CJO: 107311738(ECHDC1)
APLA: 101804132(ECHDC1)
ACYG: 106032483(ECHDC1)
TGU: 100227322(ECHDC1)
GFR: 102044218(ECHDC1)
FAB: 101806230(ECHDC1)
PHI: 102113663(ECHDC1)
PMAJ: 107201912(ECHDC1)
CCAE: 111927236(ECHDC1)
CCW: 104690802(ECHDC1)
FPG: 101918396(ECHDC1)
FCH: 102055851(ECHDC1)
CLV: 102084911(ECHDC1)
EGZ: 104131447 104131979(ECHDC1)
AAM: 106497960(ECHDC1)
ASN: 102383062(ECHDC1)
AMJ: 102560692(ECHDC1)
PSS: 102451093(ECHDC1)
CMY: 102945116(ECHDC1)
CPIC: 101939758(ECHDC1)
ACS: 100552293(echdc1)
PVT: 110077561(ECHDC1)
PBI: 103055176(ECHDC1)
GJA: 107121808(ECHDC1)
XLA: 496330(echdc1.L)
XTR: 496886(echdc1)
NPR: 108795294(ECHDC1)
DRE: 553585(echdc1)
SANH: 107688988(echdc1)
CCAR: 109098239(echdc1)
IPU: 108270580(echdc1)
AMEX: 103032922(echdc1)
TRU: 101067683(echdc1)
LCO: 104926210(echdc1)
NCC: 104955220(echdc1)
MZE: 101482663(echdc1)
OLA: 101165254(echdc1)
XMA: 102231312(echdc1)
PRET: 103478333(echdc1)
NFU: 107378790(echdc1)
KMR: 108232210(echdc1)
CSEM: 103388602(echdc1)
LCF: 108888597(echdc1)
SDU: 111227797(echdc1)
BPEC: 110171745(echdc1)
MALB: 109958274(echdc1)
ELS: 105023514(echdc1)
SFM: 108937328(echdc1)
LCM: 102352882(ECHDC1)
CMK: 103182298(echdc1)
SPU: 584418
APLC: 110980172
TCA: 659052
NVL: 108560577
DNX: 107166647
CLEC: 106664392
ZNE: 110830973
FCD: 110851854
TUT: 107363239
CEL: CELE_C32E8.9(C32E8.9)
CBR: CBG12007
CRG: 105325046
MYI: 110456022
OBI: 106867393
EPA: 110242745
ADF: 107335744
HMG: 100209361
SPAR: SPRG_12932
 » show all
Taxonomy
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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