EC: 6.4.1.4

methylcrotonoyl-CoA carboxylase (3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming))

enzyme lambda metabolic reaction experiment
uniprot:B6SKB7 [Mg2+]

ATP + Hydrogencarbonate + 3_Methylcrotonyl_CoA -> trans_3_Methylglutaconyl_CoA + Phosphate + ADP
(( (Vmax * A) ) / (Km_A + A)) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

Hydrogencarbonate + ATP + 3_Methylcrotonyl_CoA -> Phosphate + trans_3_Methylglutaconyl_CoA + ADP
(( (Vmax * C) ) / (Km_C + C)) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

ATP + Hydrogencarbonate + 3_Methylcrotonyl_CoA -> trans_3_Methylglutaconyl_CoA + ADP + Phosphate
(( (Vmax * B) ) / (Km_B + B)) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

Hydrogencarbonate + 3_Methylcrotonyl_CoA + ATP -> trans_3_Methylglutaconyl_CoA + ADP + Phosphate
(( (Vmax * (S ^ h)) ) / (( (S_half ^ h) ) + (S ^ h))) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

ATP + Hydrogencarbonate + 3_Methylcrotonyl_CoA -> trans_3_Methylglutaconyl_CoA + ADP + Phosphate
(( (Vmax * (S ^ h)) ) / (( (S_half ^ h) ) + (S ^ h))) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

ATP + Hydrogencarbonate + 3_Methylcrotonyl_CoA -> Phosphate + trans_3_Methylglutaconyl_CoA + ADP
(( (Vmax * (S ^ h)) ) / (( (S_half ^ h) ) + (S ^ h))) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37
uniprot:B6SKB7 [Mg2+]

ATP + Crotonoyl_CoA + Hydrogencarbonate -> Phosphate + Glutaconyl_1_CoA + ADP
(( (Vmax * A) ) / (Km_A + A)) buffer: 100 mM Tricine-KOH, 1 mM DTT
PH: 8
Temperature: 37

Pathways

pathway id name
BioCyc:HUMAN_LEU-DEG2-PWY leucine degradation
BioCyc:ARA_LEU-DEG2-PWY L-leucine degradation I
BioCyc:MOUSE_PWY3DJ-6 Leucine Catabolism
BioCyc:MOUSE_LEU-DEG2-PWY leucine degradation I
BioCyc:META_LEU-DEG2-PWY L-leucine degradation I
PlantCyc:ARA_LEU-DEG2-PWY L-leucine degradation I
PlantCyc:PLANT_LEU-DEG2-PWY L-leucine degradation I
Reactome:R-HSA-9865118 Diseases of branched-chain amino acid catabolism
Reactome:R-HSA-9909438 3-Methylcrotonyl-CoA carboxylase deficiency
Reactome:R-CEL-70895 Branched-chain amino acid catabolism
Reactome:R-DDI-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-DDI-196780 Biotin transport and metabolism
Reactome:R-HSA-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-HSA-196780 Biotin transport and metabolism
Reactome:R-HSA-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-1643685 Disease
Reactome:R-MMU-1430728 Metabolism
Reactome:R-MMU-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-MMU-196780 Biotin transport and metabolism
Reactome:R-MMU-70895 Branched-chain amino acid catabolism
Reactome:R-RNO-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-RNO-196780 Biotin transport and metabolism
Reactome:R-RNO-70895 Branched-chain amino acid catabolism
Reactome:R-CEL-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-CEL-196780 Biotin transport and metabolism
Reactome:R-DDI-196854 Metabolism of vitamins and cofactors
Reactome:R-DDI-71291 Amino acid and derivative metabolism
Reactome:R-DME-1430728 Metabolism
Reactome:R-DME-196849 Metabolism of water-soluble vitamins and cofactors
Reactome:R-DME-196780 Biotin transport and metabolism
Reactome:R-DME-71291 Amino acid and derivative metabolism
Reactome:R-HSA-196854 Metabolism of vitamins and cofactors
Reactome:R-HSA-3296482 Defects in vitamin and cofactor metabolism
Reactome:R-HSA-3371599 Defective HLCS causes multiple carboxylase deficiency
Reactome:R-MMU-196854 Metabolism of vitamins and cofactors
Reactome:R-RNO-196854 Metabolism of vitamins and cofactors
Reactome:R-CEL-71291 Amino acid and derivative metabolism
Reactome:R-HSA-71291 Amino acid and derivative metabolism
Reactome:R-HSA-5668914 Diseases of metabolism
Reactome:R-MMU-71291 Amino acid and derivative metabolism
Reactome:R-RNO-1430728 Metabolism
Reactome:R-RNO-71291 Amino acid and derivative metabolism
Reactome:R-CEL-1430728 Metabolism
Reactome:R-CEL-196854 Metabolism of vitamins and cofactors
Reactome:R-DDI-1430728 Metabolism
Reactome:R-DDI-70895 Branched-chain amino acid catabolism
Reactome:R-DME-196854 Metabolism of vitamins and cofactors
Reactome:R-DME-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-1430728 Metabolism
Reactome:R-HSA-3323169 Defects in biotin (Btn) metabolism
WikiPathways:WP5031 Biotin metabolism, including IMDs
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
PathBank:SMP0120662 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120806 3-Hydroxyisobutyric Aciduria
PathBank:SMP0000032 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0000173 beta-Ketothiolase Deficiency
PathBank:SMP0000199 Maple Syrup Urine Disease
PathBank:SMP0000384 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000141 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0000524 Isovaleric Acidemia
PathBank:SMP0002433 Leucine Degradation
PathBank:SMP0120661 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120665 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120745 Methylmalonic Aciduria
PathBank:SMP0120757 Propionic Acidemia
PathBank:SMP0120805 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0000138 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0000140 3-Methylglutaconic Aciduria Type III
PathBank:SMP0000238 Isovaleric Aciduria
PathBank:SMP0000523 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120660 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120664 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120736 Maple Syrup Urine Disease
PathBank:SMP0120808 Isovaleric Acidemia
PathBank:SMP0000137 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000237 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0000200 Methylmalonic Aciduria
PathBank:SMP0000521 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0087480 Propanoate Metabolism
PathBank:SMP0087516 Threonine and 2-Oxobutanoate Degradation
PathBank:SMP0120663 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120679 beta-Ketothiolase Deficiency
PathBank:SMP0120723 Isovaleric Aciduria
PathBank:SMP0120743 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120807 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000236 Propionic Acidemia
PathBank:SMP0000139 3-Methylglutaconic Aciduria Type I
PathBank:SMP0000522 3-Hydroxyisobutyric Aciduria
PathBank:SMP0087327 Valine, Leucine, and Isoleucine Degradation