Predicted to enable ATP binding activity; ligase activity; and metal ion binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be involved in L-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); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; INTERACTS WITH (+)-schisandrin B; 2,3,7,8-tetrachlorodibenzodioxine; 2,4-dinitrotoluene.
[Estradiol co-treated with Tetrachlorodibenzodioxin] results in increased expression of MCCC1 mRNA and [Estradiol co-treated with TGFB1 protein] results in decreased expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA
[diethyl phthalate co-treated with Diethylhexyl Phthalate co-treated with diisononyl phthalate co-treated with Dibutyl Phthalate co-treated with diisobutyl phthalate co-treated with butylbenzyl phthalate] affects the expression of MCCC1 mRNA