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MCCC1
Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. Note: This description may include information from UniProtKB.
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| Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4; Ligase; Mitochondrial |
| Chromosomal Location of Human Ortholog: 3q27.1 |
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Cellular Component:
3-methylcrotonyl-CoA carboxylase complex, mitochondrial; cytosol; methylcrotonoyl-CoA carboxylase complex; mitochondrial matrix; mitochondrion
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Molecular Function:
ATP binding; biotin binding; biotin carboxylase activity; metal ion binding; protein binding
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Biological Process:
biotin metabolic process; branched-chain amino acid catabolic process; leucine catabolic process; protein heterooligomerization
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Disease: 3-methylcrotonyl-coa Carboxylase 1 Deficiency
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Reference #:
Q96RQ3
(UniProtKB)
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Alt. Names/Synonyms: 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; DKFZp686B20267; FLJ25545; MCC-B; MCCA; MCCase subunit alpha; MCCC1; methylcrotonoyl-CoA carboxylase 1; methylcrotonoyl-CoA carboxylase 1 (alpha); methylcrotonoyl-CoA carboxylase alpha; Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
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Gene Symbols: MCCC1
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Molecular weight:
80,473 Da
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Basal Isoelectric point:
7.66
Predict pI for various phosphorylation states
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