hu.MAP 3.0: Complex View
Human Protein Complex Map
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Complex: huMAP3_06733.1
Complex Portal: CPX-6236
Confidence: Very High  
Proteins| Genename | Protein Name | Uniprot Annotation Score | Links |
|---|---|---|---|
| MCCC2 | Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta) | 5 | UniProt   NCBI |
| MCCC1 | Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCase subunit alpha) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 1) (3-methylcrotonyl-CoA carboxylase biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha) | 5 | UniProt   NCBI |
Enrichments
| Term ID | Corrected Pval | Fraction Complex Coverage | Proteins | Term Name |
|---|---|---|---|---|
|   CORUM:7190 | 1.70876175213e-06 | 1.0 | MCCC1 MCCC2 | 3-methylcrotonyl-CoA carboxylase |
|   WP:WP5031 | 1.53229326573e-05 | 1.0 | MCCC1 MCCC2 | Biotin metabolism including IMDs |
|   HP:0008281 | 2.05051410255e-05 | 1.0 | MCCC1 MCCC2 | Acute hyperammonemia |
|   HP:0004357 | 3.41752350426e-05 | 1.0 | MCCC1 MCCC2 | Abnormal circulating leucine concentration |
|   REAC:R-HSA-3371599 | 3.58839967947e-05 | 1.0 | MCCC1 MCCC2 | Defective HLCS causes multiple carboxylase deficiency |
|   REAC:R-HSA-3323169 | 4.78453290596e-05 | 1.0 | MCCC1 MCCC2 | Defects in biotin (Btn) metabolism |
|   REAC:R-HSA-196780 | 9.39818963671e-05 | 1.0 | MCCC1 MCCC2 | Biotin transport and metabolism |
|   KEGG:00280 | 0.00016825963378 | 1.0 | MCCC1 MCCC2 | Valine, leucine and isoleucine degradation |
|   HP:0010892 | 0.000187963792734 | 1.0 | MCCC1 MCCC2 | Abnormal circulating branched chain amino acid concentration |
|   REAC:R-HSA-3296482 | 0.000261440548076 | 1.0 | MCCC1 MCCC2 | Defects in vitamin and cofactor metabolism |
|   REAC:R-HSA-70895 | 0.000358839967947 | 1.0 | MCCC1 MCCC2 | Branched-chain amino acid catabolism |
|   GO:0004485 | 0.000468200720083 | 1.0 | MCCC1 MCCC2 | methylcrotonoyl-CoA carboxylase activity |
|   GO:1905202 | 0.000468200720083 | 1.0 | MCCC1 MCCC2 | methylcrotonoyl-CoA carboxylase complex |
|   GO:0002169 | 0.000468200720083 | 1.0 | MCCC1 MCCC2 | 3-methylcrotonyl-CoA carboxylase complex, mitochondrial |
|   HP:0002919 | 0.00138751454273 | 1.0 | MCCC1 MCCC2 | Ketonuria |
|   HP:0002179 | 0.00323297723503 | 1.0 | MCCC1 MCCC2 | Opisthotonus |
|   GO:0006552 | 0.00468200720083 | 1.0 | MCCC1 MCCC2 | leucine catabolic process |
|   GO:0016421 | 0.00702301080125 | 1.0 | MCCC1 MCCC2 | CoA carboxylase activity |
|   REAC:R-HSA-196849 | 0.00845837067304 | 1.0 | MCCC1 MCCC2 | Metabolism of water-soluble vitamins and cofactors |
|   HP:0001987 | 0.00898125176919 | 1.0 | MCCC1 MCCC2 | Hyperammonemia |
|   GO:0016885 | 0.00983221512175 | 1.0 | MCCC1 MCCC2 | ligase activity, forming carbon-carbon bonds |
|   GO:0006551 | 0.00983221512175 | 1.0 | MCCC1 MCCC2 | leucine metabolic process |
|   HP:0033107 | 0.0133830220427 | 1.0 | MCCC1 MCCC2 | Abnormal circulating proteinogenic amino acid concentration |
|   HP:0001992 | 0.0139947587499 | 1.0 | MCCC1 MCCC2 | Organic aciduria |
|   HP:0001259 | 0.0139947587499 | 1.0 | MCCC1 MCCC2 | Coma |
|   HP:0001531 | 0.0183042558888 | 1.0 | MCCC1 MCCC2 | Failure to thrive in infancy |
|   HP:0012072 | 0.0201155433461 | 1.0 | MCCC1 MCCC2 | Aciduria |
|   HP:0032943 | 0.0208639809935 | 1.0 | MCCC1 MCCC2 | Abnormal urine pH |
|   REAC:R-HSA-196854 | 0.0209254964166 | 1.0 | MCCC1 MCCC2 | Metabolism of vitamins and cofactors |
|   REAC:R-HSA-5668914 | 0.0297170756313 | 1.0 | MCCC1 MCCC2 | Diseases of metabolism |
|   HP:0001942 | 0.0332525036964 | 1.0 | MCCC1 MCCC2 | Metabolic acidosis |
|   HP:0003112 | 0.0371758206793 | 1.0 | MCCC1 MCCC2 | Abnormal circulating amino acid concentration |
|   HP:0002157 | 0.0376816141579 | 1.0 | MCCC1 MCCC2 | Azotemia |
|   HP:0004364 | 0.042927512737 | 1.0 | MCCC1 MCCC2 | Abnormal circulating nitrogen compound concentration |
|   HP:4000206 | 0.0499436884913 | 0.5 | MCCC1 | Reduced 3-methylcrotonyl CoA carboxylase activity in cultured fibroblasts |
|   HP:0006573 | 0.0499436884913 | 0.5 | MCCC1 | Acute hepatic steatosis |
Edges
| Protein 1 | Protein 2 | Score | ProteomeHD | Interface Overlap | Evidence |
|---|---|---|---|---|---|
|  MCCC2 |  MCCC1 | 1.0 | 0.334           |
structurally_consistent (dimer)        
|
bioplex3_HEK293     bioplex_WMM     Guru     bioplex3_WMM     boldt     Malo     fraction     boldt_WMM     treiber_WMM     |
Related Complexes
| Genename | Complexes |
|---|---|
| MCCC2 | huMAP3_06733.1 huMAP3_10525.1 huMAP3_13468.1 |
| MCCC1 | huMAP3_06733.1 huMAP3_10525.1 huMAP3_13468.1 |