ACAD8抗体

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     The image is immunohistochemistry of paraffin-embedded Human colon cancer tissue using P07742(ACAD8 Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P07742(ACAD8 Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

The ACAD8 antibody targets acyl-CoA dehydrogenase family member 8 (ACAD8), a mitochondrial enzyme involved in the catabolism of branched-chain amino acids (BCAAs), particularly valine. ACAD8 catalyzes the oxidation of isobutyryl-CoA to methylacrylyl-CoA, a critical step in valine degradation. Mutations in the ACAD8 gene are linked to isobutyryl-CoA dehydrogenase deficiency, an autosomal recessive disorder characterized by metabolic acidosis, developmental delays, and cardiomyopathy, though some individuals remain asymptomatic.

ACAD8 antibodies are essential tools for studying the expression, localization, and function of this enzyme in research models. They are commonly used in techniques like Western blotting, immunofluorescence, and immunohistochemistry to assess ACAD8 protein levels in tissues or cultured cells, aiding in the diagnosis and mechanistic investigation of ACAD8-related disorders. These antibodies are often raised in hosts such as rabbits or mice, with specificity validated via knockout controls or siRNA knockdown experiments.

Research employing ACAD8 antibodies has advanced understanding of mitochondrial fatty acid β-oxidation pathways and their role in metabolic diseases. Additionally, they contribute to exploring potential therapeutic strategies for inborn errors of metabolism linked to ACAD8 dysfunction.