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: 
CFA: 
609850(ECHDC1)
AML: 
100469062(ECHDC1)
UMR: 
103663427(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)
ECB: 
100073045(ECHDC1)
EPZ: 
103567873(ECHDC1)
EAI: 
MYB: 
102258272(ECHDC1)
MYD: 
102751783(ECHDC1)
HAI: 
109392627(ECHDC1)
RSS: 
109446256(ECHDC1)
PALE: 
102893310(ECHDC1)
LAV: 
100657207(ECHDC1)
MDO: 
100030825(ECHDC1)
SHR: 
100919338(ECHDC1)
GGA: 
769021(ECHDC1)
MGP: 
CJO: 
107311738(ECHDC1)
APLA: 
101804132(ECHDC1)
ACYG: 
106032483(ECHDC1)
TGU: 
100227322(ECHDC1)
GFR: 
102044218(ECHDC1)
FAB: 
101806230(ECHDC1)
PHI: 
102113663(ECHDC1)
PMAJ: 
107201912(ECHDC1)
CCW: 
104690802(ECHDC1)
FPG: 
101918396(ECHDC1)
FCH: 
102055851(ECHDC1)
CLV: 
102084911(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)
SRX: 
SANH: 
107688988(echdc1)
SGH: 
IPU: 
108270580(echdc1)
TRU: 
101067683(echdc1)
TNG: 
LCO: 
104926210(echdc1)
NCC: 
104955220(echdc1)
MZE: 
101482663(echdc1)
OLA: 
101165254(echdc1)
XMA: 
102231312(echdc1)
CSEM: 
103388602(echdc1)
LCF: 
108888597(echdc1)
HCQ: 
BPEC: 
110171745(echdc1)
SASA: 
ELS: 
105023514(echdc1)
SFM: 
108937328(echdc1)
LCM: 
102352882(ECHDC1)
CMK: 
103182298(echdc1)
BFO: 
SPU: 
TCA: 
NVL: 
API: 
DNX: 
PHU: 
DPX: 
ISC: 
TUT: 
CEL: 
CELE_C32E8.9(C32E8.9)
CBR: 
NAI: 
HRO: 
LGI: 
CRG: 
OBI: 
NVE: 
ADF: 
TAD: 
SRE: 
PIF: 
PSOJ: 
SPAR: 
 » 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 (2011) 42992-3003.
  Sequence
[hsa:55862] [mmu:52665] [rno:361465]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 

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