GCDH抗体—艾普蒂

+ +

•  

   

     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-3: K562, 293T and NIH/3T3 cell lysates, Primary antibody: P02718(GCDH Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 2 minutes    

  
  

The GCDH (Glutaryl-CoA Dehydrogenase) antibody is a crucial tool for studying the enzyme encoded by the GCDH gene, which plays a vital role in mitochondrial amino acid metabolism. GCDH catalyzes the oxidation of glutaryl-CoA to crotonyl-CoA in the degradation pathways of lysine, hydroxylysine, and tryptophan. Deficiencies in GCDH activity are linked to glutaric acidemia type 1 (GA1), an autosomal recessive disorder characterized by toxic accumulation of glutaric acid and 3-hydroxyglutaric acid, leading to neurological damage, particularly in infancy.

GCDH antibodies are widely used in research to detect and quantify GCDH protein expression in tissues or cell lines, aiding in the study of GA1 pathogenesis, enzyme function, and metabolic dysregulation. These antibodies are essential for techniques like Western blotting, immunohistochemistry, and immunofluorescence, enabling localization of GCDH within mitochondria. They also support diagnostic applications, including confirming suspected GA1 cases through protein expression analysis.

Structurally, GCDH is a homotetramer, and antibodies targeting specific epitopes help identify mutations or post-translational modifications affecting enzyme stability or activity. Species-specific GCDH antibodies (e.g., human, mouse) facilitate cross-species comparative studies. Ongoing research leverages these antibodies to explore therapeutic strategies, such as gene therapy or enzyme replacement, to address GCDH deficiency. Overall, GCDH antibodies are indispensable for advancing both basic and clinical insights into mitochondrial metabolism and related disorders.