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3-methylcrotonyl-CoA + ATP + HCO3-
3-methylglutaconyl-CoA + ADP + phosphate
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mitochondrial leucine catabolic pathway
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ATP + 3-methylcrotonoyl-CoA + HCO3-
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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 + phosphate + 3-methylglutaconyl-CoA
ATP + acetyl-CoA + HCO3-
ADP + malonyl-CoA + phosphate
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r
additional information
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ATP + 3-methylcrotonoyl-CoA + HCO3-
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induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
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ATP + 3-methylcrotonoyl-CoA + HCO3-
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enzyme of the leucine oxidative pathway
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ATP + 3-methylcrotonoyl-CoA + HCO3-
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induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
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ATP + 3-methylcrotonoyl-CoA + HCO3-
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induction with 3-methylcrotonoyl-CoA as growth substrate. No activity with 3-methylpentanoate as growth substrate
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ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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a step in the metabolism of leucine into 3-methylcrotonyl CoA and related metabolites, overview
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ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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ATP + 3-methylcrotonoyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
enzyme is involved in leucine catabolic pathway
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + 3-methylglutaconyl-CoA + phosphate
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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key enzyme in the fermentation of glutamate to ammonia, carbon dioxide, hydrogen, acetate and butyrate
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r
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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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
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
pivotal reaction required for both leucine catabolism and isoprenoid metabolism
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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catalyzes essential steps in the metabolism of amino acids
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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irreversible catabolic pathway of leucine
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ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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irreversible catabolic pathway of leucine
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
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in addition to leucine catabolism required for the operation of the mevalonate shunt
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ir
additional information
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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
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additional information
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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
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additional information
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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
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additional information
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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
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