Anti-MCCC2 Rabbit Polyclonal Antibody for WB, IHC-P, ELISA - Q9HCC0

Core Product Specifications and Parameters

Biological Background: MCCC2 Function and Localization

• MCCC2, also known as MCCB or MCCCbeta, encodes the beta chain of 3-methylcrotonyl-CoA carboxylase, a biotin-independent mitochondrial enzyme.
• It serves as the carboxyltransferase subunit, catalyzing the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, an essential step in leucine and isovaleric acid catabolism.
• The protein localizes to the mitochondrial matrix, consistent with its metabolic role in branched-chain amino acid degradation.
• Multiple post-translational modifications, including acetylation, and alternative splicing have been documented, potentially modulating its activity and generating diverse isoforms.
• Defects in MCCC2 are linked to 3-methylcrotonyl-CoA carboxylase deficiency, an inborn error of metabolism characterized by organic aciduria.
• As an ATP-binding ligase, MCCC2 requires ATP for the carboxylation reaction, highlighting its role in energy-dependent metabolic pathways.

Experimental Guidance and Technical Tips

• For Western blot, a band corresponding to the predicted molecular weight of ~61 kDa should be observed in samples from human, mouse, or rat tissues.
• For immunohistochemistry (IHC-P), consider antigen retrieval methods and validate the antibody in appropriate control tissues to confirm specificity.
• Ensure proper storage upon receipt (shipped on ice bags) and aliquot to avoid repeated freeze-thaw cycles.
• As with any polyclonal antibody, lot-to-lot variability may occur; it is advisable to test each new lot in the intended application.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio offers a comprehensive portfolio of IVD raw materials, including validated antibodies like this MCCC2 polyclonal antibody, to support research on leucine metabolism and mitochondrial disorders. We provide one-stop access to bulk ancillary reagents, optimized antibody pairs, and rare target sourcing, streamlining your procurement from concept to clinic. Partner with us to accelerate your diagnostic and laboratory workflows with reliable materials.