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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

Reaction

ATP
+
3-methylcrotonoyl-CoA
 +
HCO3-
 +
H+
=
ADP
 +
phosphate
 +
3-methylglutaconyl-CoA

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 Ligases
         6.4 Forming carbon-carbon bonds
             6.4.1 Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
                6.4.1.4 methylcrotonoyl-CoA carboxylase

Engineering

Engineering on EC 6.4.1.4 - methylcrotonoyl-CoA carboxylase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
M169K
-
mutant with lack of carboxylation activity
R338Q
-
mutant with lack of carboxylation activity
R338S
-
mutant with lack of carboxylation activity
R385S
-
human mutant leading to methylcrotonylglycinuria
G46E
-
the mutation of the MCCA gene is associated with methylcrotonylglycinuria
G517R
-
the mutation of the MCCB gene is associated with methylcrotonylglycinuria
L355F
-
the mutation of the MCCB gene is associated with methylcrotonylglycinuria
Q477R
-
the mutation of the MCCB gene is associated with methylcrotonylglycinuria
R385S
-
mutant leading to biochemical abnormalities and clinical symptoms in heterozygous individuals
Y520S
-
the mutation of the MCCB gene is associated with methylcrotonylglycinuria
F417Y
beta-subunit mutant retains activity, kcat/Km increased compared to wild-type, kcat (3-methylcrotonoyl-CoA) increased compared to wild-type, Km (3-methylcrotonoyl-CoA) increased compared to wild-type
F417Y/G423A
beta-subunit mutant retains activity, kcat/Km similar to wild-type, kcat (3-methylcrotonoyl-CoA) increased compared to wild-type, Km (3-methylcrotonoyl-CoA) increased compared to wild-type
G423A
beta-subunit mutant retains activity, kcat/Km decreased compared to wild-type, kcat (3-methylcrotonoyl-CoA) decreased compared to wild-type, Km (3-methylcrotonoyl-CoA) slightly decreased compared to wild-type
R51A
beta-subunit mutant retains activity, kcat/Km decreased compared to wild-type, kcat (3-methylcrotonoyl-CoA) decreased compared to wild-type, Km (3-methylcrotonoyl-CoA) slightly increased compared to wild-type
S187A
beta-subunit mutant shows no enzymatic activity
Y422D
beta-subunit mutant shows no enzymatic activity
additional information
-
the IVS7-1GNA splice site mutation of the MCCB gene is associated with methylcrotonylglycinuria and shows 31fold reduced activity compared to the wild type