MGP Database

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MGP005568

Ontology/Pathway Information

Entrez Gene ID56922
Gene Namemethylcrotonoyl-CoA carboxylase 1 (alpha)
Gene Symbol MCCC1
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005829 TAS:ReactomeCcytosol
GO:0005743 IEA:EnsemblCmitochondrial inner membrane
GO:0005759 NAS:UniProtKBCmitochondrial matrix
GO:0005739 IDA:HPACmitochondrion
GO:0005524 IEA:UniProtKB-KWFATP binding
GO:0009374 NAS:UniProtKBFbiotin binding
GO:0004075 IEA:InterProFbiotin carboxylase activity
GO:0046872 IEA:InterProFmetal ion binding
GO:0004485 IDA:UniProtKBFmethylcrotonoyl-CoA carboxylase activity
GO:0006768 NAS:UniProtKBPbiotin metabolic process
GO:0009083 TAS:ReactomePbranched-chain amino acid catabolic process
GO:0034641 TAS:ReactomePcellular nitrogen compound metabolic process
GO:0006552 NAS:UniProtKBPleucine 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_11153Biotin transport and metabolism
REACT_197Branched-chain amino acid catabolism
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_111217Metabolism
REACT_13Metabolism of amino acids and derivatives
REACT_11193Metabolism of vitamins and cofactors
REACT_11238Metabolism of water-soluble vitamins and cofactors
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
SMP00199Maple Syrup Urine Disease
SMP00384Methylmalonate Semialdehyde Dehydrogenase Deficiency
SMP00200Methylmalonic Aciduria
SMP00236Propionic Acidemia
SMP00032Valine, Leucine and Isoleucine Degradation
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