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MUT Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM). MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Belongs to the methylmalonyl-CoA mutase family. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC; Isomerase; Mitochondrial
Chromosomal Location of Human Ortholog: 6p12.3
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: cobalamin binding; metal ion binding; methylmalonyl-CoA mutase activity
Biological Process: cobalamin metabolic process; homocysteine metabolic process; post-embryonic development; short-chain fatty acid catabolic process
Disease: Methylmalonic Aciduria Due To Methylmalonyl-coa Mutase Deficiency
Reference #:  P22033 (UniProtKB)
Alt. Names/Synonyms: MCM; methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase, mitochondrial; MUT; MUTA
Gene Symbols: MUT
Molecular weight: 83,134 Da
Basal Isoelectric point: 6.48  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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