mccc-1 encodes an ortholog of the human gene ALPHA METHYLCROTONOYL-COENZYME A CARBOXYLASE 1 (MCCC1; 3-MCC), which when mutated leads to beta-methylcrotonyl-glycinuria (OMIM:210200).
Predicted to enable ATP binding activity and ligase activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be located in mitochondrion. Human ortholog(s) of this gene implicated in 3-Methylcrotonyl-CoA carboxylase 1 deficiency. Is an ortholog of human MCCC1 (methylcrotonyl-CoA carboxylase subunit 1).
Map position created from combination of previous interpolated map position (based on known location of sequence) and allele information. Therefore this is not a genetic map position based on recombination frequencies or genetic experiments. This was done on advice of the CGC.