6.4.1.4: methylcrotonoyl-CoA carboxylase
This is an abbreviated version!
For detailed information about methylcrotonoyl-CoA carboxylase, go to the full flat file.
Word Map on EC 6.4.1.4
-
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
- 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
Reaction
Synonyms
3-MCC, 3-methylcrotonyl CoA carboxylase, 3-methylcrotonyl coenzyme A carboxylase, 3-methylcrotonyl coenzyme-A carboxylase, 3-methylcrotonyl-CoA carboxylase, 3-methylcrotonyl-CoA:carbon-dioxide ligase (ADP-forming), 3-methylcrotonyl-coenzyme A, 3-methylcrotonyl-coenzyme A carboxylase, beta-MCC, beta-methylcrotonyl CoA carboxylase, beta-Methylcrotonyl coenzyme A carboxylase, beta-methylcrotonyl-CoA carboxylase, Carboxylase, methylcrotonyl coenzyme A, glutaconyl-CoA decarboxylase, glutaconyl-CoA:biotin carboxytransferase, liuB, liuD, MC-CoA carboxylase, Mcase, MCC, Mcc2, MCCA, MCCase, MCCB, MCCC, Methylcrotonyl coenzyme A carboxylase, methylcrotonyl-CoA carboxylase, Pa-MCCase, PaMCC
ECTree
6.4.1.4 methylcrotonoyl-CoA carboxylaseAdvanced search results
results (
results (Natural Substrates Products
Natural Substrates Products on EC 6.4.1.4 - methylcrotonoyl-CoA carboxylase
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
top
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
-
?
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
-
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
enzyme is involved in leucine catabolic pathway
-
-
?
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
-
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
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
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
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
-
catalyzes essential steps in the metabolism of amino acids
-
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
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
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
-
irreversible catabolic pathway of leucine
-
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
-
in addition to leucine catabolism required for the operation of the mevalonate shunt
-
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
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
-
-
?
