MGP Database

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MGP001986

Ontology/Pathway Information

Entrez Gene ID4594
Gene Namemethylmalonyl CoA mutase
Gene Symbol MUT
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005759 TAS:ReactomeCmitochondrial matrix
GO:0005739 TAS:ProtIncCmitochondrion
GO:0031419 IEA:UniProtKB-KWFcobalamin binding
GO:0046872 IEA:UniProtKB-KWFmetal ion binding
GO:0004494 TAS:ProtIncFmethylmalonyl-CoA mutase activity
GO:0072341 IDA:UniProtKBFmodified amino acid binding
GO:0044255 TAS:ReactomePcellular lipid metabolic process
GO:0009235 TAS:ReactomePcobalamin metabolic process
GO:0006635 TAS:ReactomePfatty acid beta-oxidation
GO:0050667 IDA:UniProtKBPhomocysteine metabolic process
GO:0009791 IEA:EnsemblPpost-embryonic development
GO:0019626 TAS:ReactomePshort-chain fatty acid catabolic process
GO:0044281 TAS:ReactomePsmall molecule metabolic process
GO:0006766 TAS:ReactomePvitamin metabolic process
GO:0006767 TAS:ReactomePwater-soluble vitamin metabolic process
REACTOME Pathway Links
REACTOME Pathway IDDescription
REACT_163862Cobalamin (Cbl, vitamin B12) transport and metabolism
REACT_169280Defective AMN causes hereditary megaloblastic anemia 1
REACT_169403Defective BTD causes biotidinase deficiency
REACT_169178Defective CD320 causes methylmalonic aciduria
REACT_169132Defective CUBN causes hereditary megaloblastic anemia 1
REACT_169415Defective GIF causes intrinsic factor deficiency
REACT_169312Defective HLCS causes multiple carboxylase deficiency
REACT_169363Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF
REACT_169316Defective MMAA causes methylmalonic aciduria type cblA
REACT_169318Defective MMAB causes methylmalonic aciduria type cblB
REACT_169169Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC
REACT_169256Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD
REACT_169149Defective MTR causes methylmalonic aciduria and homocystinuria type cblG
REACT_169439Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE
REACT_169313Defective MUT causes methylmalonic aciduria mut type
REACT_169120Defective TCN2 causes hereditary megaloblastic anemia
REACT_169238Defects in biotin (Btn) metabolism
REACT_169429Defects in cobalamin (B12) metabolism
REACT_169385Defects in vitamin and cofactor metabolism
REACT_116125Disease
REACT_22279Fatty acid, triacylglycerol, and ketone body metabolism
REACT_111217Metabolism
REACT_22258Metabolism of lipids and lipoproteins
REACT_11193Metabolism of vitamins and cofactors
REACT_11238Metabolism of water-soluble vitamins and cofactors
REACT_1473Mitochondrial Fatty Acid Beta-Oxidation
REACT_993Propionyl-CoA catabolism
SMP Pathway Links
SMP IDDescription
SMP001372-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
SMP001383-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
SMP005213-hydroxyisobutyric acid dehydrogenase deficiency
SMP005223-hydroxyisobutyric aciduria
SMP002373-Methylcrotonyl Coa Carboxylase Deficiency Type I
SMP001393-Methylglutaconic Aciduria Type I
SMP001403-Methylglutaconic Aciduria Type III
SMP001413-Methylglutaconic Aciduria Type IV
SMP00173Beta-Ketothiolase Deficiency
SMP00523Isobutyryl-coa dehydrogenase deficiency
SMP00524Isovaleric acidemia
SMP00238Isovaleric Aciduria
SMP00198Malonic Aciduria
SMP00502Malonyl-coa decarboxylase deficiency
SMP00199Maple Syrup Urine Disease
SMP00384Methylmalonate Semialdehyde Dehydrogenase Deficiency
SMP00200Methylmalonic Aciduria
SMP00201Methylmalonic Aciduria Due to Cobalamin-Related Disorders
SMP00016Propanoate Metabolism
SMP00236Propionic Acidemia
SMP00452Threonine and 2-Oxobutanoate Degradation
SMP00032Valine, Leucine and Isoleucine Degradation
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