Activating Compound | Comment | Organism | Structure |
---|---|---|---|
hMMAA | hMCM loses activity during catalysis, but the interaction with human MMAA (hMMAA), a GTPase protein, avoids this loss or restores hMCM activity. Formation and accumulation of OH2Cbl, the oxidized form of cofactor AdoCbl formed during catalysis, is the cause of hMCM inactivation. The complex formation of hMCM/hMMAA decreases the rate of oxidized cofactor formation, protecting the hMCM enzyme. hMMAA is able to remove the damaged cofactor through GTP hydrolysis.a modification in the kinetic parameters of hMCM in presence of hMMAA is observed. No activity is recovered in the presence of hMMAAwith GMPPNP, confirming the need of GTP hydrolysis for this cofactor removal. In vivo localization of hMMAA and its colocalization with hMCM in human fibroblasts mitochondria. hMMAAis localized in both the cytoplasm and mitochondria but in less abundance and distribution than enzyme hMCM | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | Michaelis-Menten kinetics | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytoplasm | enzyme hMCM preferably locates in mitochondria, with small amounts of protein in the cytoplasm | Homo sapiens | 5737 | - |
mitochondrion | enzyme hMCM preferably locates in mitochondria, with small amounts of protein in the cytoplasm | Homo sapiens | 5739 | - |
additional information | immunohistochemic analysis of subcellular localization in fibroblasts, overview | Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Mg2+ | required | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
(R)-methylmalonyl-CoA | Homo sapiens | - |
succinyl-CoA | - |
r |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P22033 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
GMO2784 cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
(R)-methylmalonyl-CoA | - |
Homo sapiens | succinyl-CoA | - |
r |
Synonyms | Comment | Organism |
---|---|---|
hMCM | - |
Homo sapiens |
mitochondrial methylmalonyl-CoA mutase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
20 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7.5 | - |
assay at | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
adenosylcobalamin | AdoCbl, the enzyme converts methylmalonyl-CoA to succinyl-CoA employing highly reactive radicals from its cofactor adenosylcobalamin to perform its reaction. Formation and accumulation of OH2Cbl, the oxidized form of the AdoCbl cofactor formed during catalysis, is the cause of hMCM inactivation. GTPase hMMAA is able to remove the damaged cofactor through GTP hydrolysis | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | human mitochondrial methylmalonyl-CoA mutase (hMCM) is an isomerase that converts methylmalonyl-CoA to succinyl-CoA, a crucial step for the incorporation of some compounds derived from the diet into the central metabolism. hMCM employs highly reactive radicals from its cofactor (adenosylcobalamin, AdoCbl) to perform its reaction. hMCM loses activity during catalysis, but the interaction with human MMAA (hMMAA), a GTPase protein, avoids this loss or restores hMCM activity. Formation and accumulation of OH2Cbl, the oxidized form of cofactor AdoCbl formed during catalysis, is the cause of hMCM inactivation. The complex formation of hMCM/hMMAA decreases the rate of oxidized cofactor formation, protecting the hMCM enzyme. hMMAA is able to remove the damaged cofactor through GTP hydrolysis.a modification in the kinetic parameters of hMCM in presence of hMMAA is observed | Homo sapiens |