Information on EC 4.1.1.94 - ethylmalonyl-CoA decarboxylase

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The enzyme appears in viruses and cellular organisms

EC NUMBER
COMMENTARY hide
4.1.1.94
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RECOMMENDED NAME
GeneOntology No.
ethylmalonyl-CoA decarboxylase
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REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
(S)-ethylmalonyl-CoA = butanoyl-CoA + CO2
show the reaction diagram
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SYSTEMATIC NAME
IUBMB Comments
(S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming)
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).
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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Manually annotated by BRENDA team
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Manually annotated by BRENDA team
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Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
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enzyme mutation may be involved in the development of certain forms of ethylmalonic aciduria
metabolism
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ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
physiological function
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ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
additional information
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ECHDC1-specific siRNAs decrease the ethylmalonyl-CoA decarboxylase activity in human cells and increase the formation of ethylmalonate, most particularly in cells incubated with butyrate
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
show the reaction diagram
methylmalonyl-CoA
propanoyl-CoA + CO2
show the reaction diagram
additional information
?
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INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
MgATP2-
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inhibits the enzyme slightly
additional information
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ECHDC1-specific siRNAs decrease the ethylmalonyl-CoA decarboxylase activity in human cells and increase the formation of ethylmalonate, most particularly in cells incubated with butyrate
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.00096 - 0.0065
(S)-ethylmalonyl-CoA
0.0031 - 0.0151
methylmalonyl-CoA
TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
9.5 - 10
(S)-ethylmalonyl-CoA
1.61 - 1.81
methylmalonyl-CoA
kcat/KM VALUE [1/mMs-1]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
1500 - 9900
(S)-ethylmalonyl-CoA
15207
110 - 510
methylmalonyl-CoA
790
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
8800
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purified recombinant ECHDC1, pH 7.1, 30°C
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
additional information
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subcellular localization analysis, overview
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Manually annotated by BRENDA team
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
34000
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x * 34000, recombinant ECHDC1, SDS-PAGE
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
?
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x * 34000, recombinant ECHDC1, SDS-PAGE
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
native enzyme from liver by anion exchange chromatography
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recombinant enzyme by metal affinity chromatography
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
ECHDC1 from mouse brain cDNA, expression as N-terminally His-tagged enzyme
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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expression of siRNA in HEK-293T cells suppresses the enzyme with an about 3fold decrease in ECHDC1 mRNA levels