Mccc2 - methylcrotonyl-CoA carboxylase subunit 2

Contributes to methylcrotonoyl-CoA carboxylase activity. Involved in coenzyme A metabolic process. Predicted to be located in mitochondrial matrix. Predicted to be part of methylcrotonoyl-CoA carboxylase complex. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in 3-Methylcrotonyl-CoA carboxylase 2 deficiency and mitochondrial metabolism disease. Orthologous to human MCCC2 (methylcrotonyl-CoA carboxylase subunit 2). [provided by Alliance of Genome Resources, Apr 2022]