| Name | 3-methylcrotonyl-CoA carboxylase complex, mitochondrial |
| ID | GO_0002169 |
| collection_id | Cellular component |
| Exact synonym | mitochondrial 3-methylcrotonyl-CoA carboxylase holoenzyme, mitochondrial MCCC complex, mitochondrial methylcrotonoyl-CoA carboxylase complex |
| Description | A mitochondrial protein complex which is capable of 3-methylcrotonyl-CoA carboxylase activity. In mammals, at least, consists as a dodecamer of 6 alpha and 6 beta subunits. MCCC-alpha has a covalently bound biotin essential for the ATP-dependent carboxylation. MCCC-beta possesses carboxyltransferase activity which presumably is essential for binding to 3-methylcrotonyl-CoA. |
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| Genes |