Mccc1 - methylcrotonyl-CoA carboxylase subunit 1

Predicted to enable ATP binding activity and metal ion binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be involved in leucine catabolic 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 1 deficiency. Orthologous to human MCCC1 (methylcrotonyl-CoA carboxylase subunit 1). [provided by Alliance of Genome Resources, Apr 2022]