PCCB抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Mouse heart tissue lysate, Primary antibody: P10599(PCCB Antibody) at dilution 1/1000, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 40 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human gastric cancer tissue using P10599(PCCB Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P10599(PCCB Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

PCCB antibodies target the beta subunit of propionyl-CoA carboxylase (PCC), a mitochondrial enzyme critical in amino acid and fatty acid metabolism. PCC is a heterodimeric enzyme composed of alpha (PCCA) and beta (PCCB) subunits, encoded by the *PCCA* and *PCCB* genes, respectively. It catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA, a key step in the catabolism of branched-chain amino acids (valine, isoleucine) and odd-chain fatty acids. Mutations in *PCCB* cause propionic acidemia (PA), an autosomal recessive disorder characterized by toxic accumulation of propionyl-CoA metabolites, leading to metabolic crises, neurological impairment, and multi-organ dysfunction.

PCCB antibodies are essential tools in research and diagnostics. They enable detection and quantification of PCCB protein levels via techniques like Western blotting, immunohistochemistry, or ELISA, aiding in the study of PA pathophysiology and enzyme expression patterns. Clinically, these antibodies assist in confirming PA diagnosis by identifying reduced PCCB expression in patient samples. They also support newborn screening programs and carrier testing. Additionally, PCCB antibodies contribute to therapeutic development, such as evaluating gene therapy or enzyme replacement strategies. Their specificity and reliability are crucial for understanding PCC's structural-functional dynamics and its role in mitochondrial metabolic pathways.