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Santa Cruz Biotechnology is a world leader in the development of products for the biomedical research market. Over the past 30+ years, the Company has focused on the ongoing development of research antibodies, siRNA and CRISPR Gene editing tools, biochemicals, labware and more recently has expanded into animal health care products. Santa Cruz Biotechnology has the highest commitment to quality and customer service.
Santa Cruz Biotechnology
MCCA (A-4) | Santa Cruz Biotechnology
mouse monoclonal IgG2a; 3-methylcrotonyl-CoA:carboxylase (MCC) is an enzyme crucial in the breakdown of the branched chain amino acid leucine. Methylcrotonoyl-CoA carboxylase α chain (MCCA), also designated 3-methylcrotonyl-CoA carboxylase 1, is located in the mitochondrial matrix. MCCA functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. MCCA has a Biotin cofactor. The gene encoding for the 725 amino acid MCCA protein maps to chromosome 3q26-q28 and consists of 19 exons. Defects in this gene are associated with 3-methylcrotonylglycinuria (MCGI), an autosomal recessive disorder characterized by muscular hypotonia and atrophy. Human MCC deficiency, also inherited recessively, is characterized by 3-methylcrotonyl-CoA accumulation. Symptoms may be highly variable, ranging from completely asymptomatic to metabolic acidosis and death in infancy.
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