MCCC1抗体—艾普蒂

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     Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-5: 293T, HepG2, A172 cell, Mouse heart tissue, Jurkat cell lysates, Primary antibody: P02941(MCCC1 Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P02941(MCCC1 Antibody) at dilution 1/45. (Original magnification: ×200)    

  
  

The MCCC1 antibody is a crucial tool for studying the methylcrotonoyl-CoA carboxylase 1 (MCCC1) protein, encoded by the *MCCC1* gene. This enzyme is a subunit of the heterodimeric 3-methylcrotonyl-CoA carboxylase (MCC), which plays a vital role in mitochondrial leucine catabolism. Specifically, MCCC1 combines with MCCC2 to form the active MCC enzyme, catalyzing the carboxylation of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA—a critical step in breaking down leucine, an essential amino acid. Mutations in *MCCC1* are linked to 3-methylcrotonyl-CoA carboxylase deficiency (MCC deficiency), an autosomal recessive disorder characterized by metabolic acidosis, hypoglycemia, and developmental delays, though some cases remain asymptomatic.

MCCC1 antibodies are widely used in research and diagnostics to detect protein expression, localization, and quantification in tissues or cell lines. They aid in studying MCC deficiency pathogenesis, validating gene-editing models, and exploring MCCC1's role in diseases like cancer, where altered leucine metabolism may influence tumor progression. These antibodies (polyclonal or monoclonal, often raised in rabbits or mice) are optimized for techniques like Western blotting, immunohistochemistry, and immunofluorescence. Their specificity and sensitivity make them valuable for investigating mitochondrial metabolic pathways, assessing enzyme activity in patient samples, and developing targeted therapies. By enabling precise detection of MCCC1. these antibodies contribute to both basic research and clinical applications, such as early diagnosis and therapeutic monitoring in metabolic disorders.