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Information on EC 6.4.1.4 - methylcrotonoyl-CoA carboxylase
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6.4.1.4 methylcrotonoyl-CoA carboxylaseIUBMB Comments
A biotinyl-protein.
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Word Map
- 6.4.1.4
- rrna
-
dna-dna
- phosphatidylethanolamine
- carboxylases
-
rod-shaped
-
chemotaxonomic
-
biotin-dependent
- diphosphatidylglycerol
- propionyl-coa
-
3-hydroxyisovaleric
-
polyphasic
-
gram-stain-negative
-
biotin-containing
- acidurias
-
aminolipids
-
3-methylcrotonylglycine
-
non-motile
-
isovaleric
-
deep-sea-derived
-
propionyl
- holocarboxylase
-
catalase-positive
- biotinidase
-
oxidase-positive
- sediminis
-
anteiso-c15:0
-
3-hydroxyisovalerylcarnitine
-
cyclo
-
gram-staining-negative
- ubiquinone-10
-
methylcitric
- xiamenensis
-
non-spore-forming
-
ll-diaminopimelic
- 3-methylglutaconyl-coa
-
biotin-responsive
- apocarboxylases
-
weihai
-
hresims
-
biotin-deficient
- rhodobacteraceae
- agriculture
- medicine
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Reaction Schemes
Synonyms
mccc, 3-methylcrotonyl-coa carboxylase, mccase, methylcrotonyl-coa carboxylase, mcase, beta-methylcrotonyl-coa carboxylase, 3-mcc, 3-methylcrotonyl-coenzyme a carboxylase, 3-methylcrotonyl coa carboxylase, beta-methylcrotonyl coa carboxylase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
3-methylcrotonyl-CoA carboxylase
3-methylcrotonyl-coenzyme A carboxylase
beta-MCC
beta-methylcrotonyl CoA carboxylase
-
-
-
-
beta-Methylcrotonyl coenzyme A carboxylase
-
-
-
-
beta-methylcrotonyl-CoA carboxylase
Carboxylase, methylcrotonyl coenzyme A
-
-
-
-
liuB
Mcase
MCC
MCCA
MCCase
MCCB
Methylcrotonyl coenzyme A carboxylase
-
-
-
-
Mcase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
ATP + 3-methylcrotonoyl-CoA + HCO3- + H+ = ADP + phosphate + 3-methylglutaconyl-CoA
ATP and HCO3- bind sequentially to the enzyme. ATP and 3-methylcrotonoyl-CoA bind in ping-pong fashion
-
ATP + 3-methylcrotonoyl-CoA + HCO3- + H+ = ADP + phosphate + 3-methylglutaconyl-CoA
bi bi uni uni ping-pong mechanism, i.e. double-displacement kinetic mechanism
-
ATP + 3-methylcrotonoyl-CoA + HCO3- + H+ = ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
carboxylation
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
-
-
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SYSTEMATIC NAME
IUBMB Comments
3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)
A biotinyl-protein.
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CAS REGISTRY NUMBER
COMMENTARY
9023-95-4
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
ATP + (2Z)-3-ethylcrotonoyl-CoA
ADP + phosphate + 3-ethylglutaconyl-CoA
-
12% of the activity relative to 3-methylcrotonoyl-CoA
-
-
?
UTP + 3-methylcrotonoyl-CoA + HCO3-
UDP + phosphate + 3-methylglutaconyl-CoA
-
at 62% of the activity relative to ATP
-
-
?
3-methylcrotonyl-CoA + ATP + HCO3-
3-methylglutaconyl-CoA + ADP + phosphate
-
-
-
?
3-methylcrotonyl-CoA + ATP + HCO3-
3-methylglutaconyl-CoA + ADP + phosphate
-
mitochondrial leucine catabolic pathway
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
?
-
induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
?
-
enzyme of the leucine oxidative pathway
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
?
-
induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
?
-
induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
r
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
the forward reaction is about 10times faster than the reverse reaction
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
a step in the metabolism of leucine into 3-methylcrotonyl CoA and related metabolites, overview
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
substrate of MCCase, EC 6.4.1.4, and GCCAse, EC 6.4.1.5
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
enzyme is involved in leucine catabolic pathway
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
?
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
-
-
-
-
?
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
-
-
-
-
?
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
-
-
-
-
?
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
-
-
-
-
?
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
r
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
key enzyme in the fermentation of glutamate to ammonia, carbon dioxide, hydrogen, acetate and butyrate
-
r
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
mitochondrial catabolism of leucine, also might function in the catabolism of isoprenoids and the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
pivotal reaction required for both leucine catabolism and isoprenoid metabolism
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
catalyzes essential steps in the metabolism of amino acids
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
r
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
irreversible catabolic pathway of leucine
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
irreversible catabolic pathway of leucine
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
-
-
ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
ir
ATP + acetoacetyl-CoA + HCO3-
?
-
12% of the activity relative to 3-methylcrotonoyl-CoA
-
-
?
ATP + acetoacetyl-CoA + HCO3-
?
-
acetoacetyl-CoA inhibits, no activity as substrate
-
-
?
ATP + crotonoyl-CoA + HCO3-
?
-
27% of the activity relative to 3-methylcrotonoyl-CoA
-
-
?
ATP + crotonoyl-CoA + HCO3-
?
-
44% of the activity relative to 3-methylcrotonoyl-CoA
-
-
?
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
ATP + crotonyl-CoA + HCO3-
ADP + phosphate + glutaconyl-CoA
-
much poorer substrate
-
ir
additional information
?
-
-
catalyzes ATP/diphosphate exchange reaction
-
-
?
additional information
?
-
-
children with 3-MCC deficiency show mental retardation or metabolic disturbances including ketoacidosis, hypoglycemia, or Reye syndrome, but 3-MCC deficiency can also occur in asymptomatic individuals or as non-specific fasting intolerance with normal neurological outcome, overview. The deficiency is diagnozed by 3-methylcrotonylglycine content in the urine
-
-
?
additional information
?
-
-
induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, biotin deficiency does cause human birth defects, overview
-
-
?
additional information
?
-
-
induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, in mice a marginal degree of biotin deficiency in the dam causes a severe degree of deficiency in the fetus, overview
-
-
?
additional information
?
-
-
geranyl-CoA carboxylase, GCCase, may substitute for MCCase in leucine catabolism, both the MCCase and GCCase enzymes play important roles in the leucine and acyclic terpene catabolic pathways, overview
-
-
?
additional information
?
-
-
MCCase shows no activity with geranyl-CoA, while the geranyl-CoA carboxylase, EC 6.4.1.5, is also active with 3-methylcrotonoyl-CoA in addition to the preferred substrate geranyl-CoA
-
-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3-methylcrotonyl-CoA + ATP + HCO3-
3-methylglutaconyl-CoA + ADP + phosphate
-
mitochondrial leucine catabolic pathway
-
?