PDSS2抗体;PDSS2 Antibody—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Hela cells, Primary antibody: P12038(PDSS2 Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 20 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P12038(PDSS2 Antibody) at dilution 1/30. (Original magnification: ×200)    

  
  

The PDSS2 (Prenyl Diphosphate Synthase, Subunit 2) antibody is primarily utilized in research to study the expression and function of the PDSS2 protein, a critical enzyme in the biosynthesis of coenzyme Q10 (CoQ10). PDSS2 encodes a subunit of decaprenyl diphosphate synthase, which catalyzes the elongation of polyprenyl chains during CoQ10 production—a vital component of the mitochondrial electron transport chain and cellular antioxidant defense. Mutations in the PDSS2 gene are linked to primary CoQ10 deficiency, a rare autosomal recessive disorder associated with severe infantile-onset conditions, including steroid-resistant nephrotic syndrome, encephalopathy, and cardiomyopathy.

In clinical and experimental settings, the PDSS2 antibody serves as a tool to detect PDSS2 protein levels in tissues or cell lines, aiding in the diagnosis of CoQ10-related disorders and mechanistic studies of mitochondrial dysfunction. Additionally, research explores its role in cancer, as altered CoQ10 metabolism may influence tumor progression. The antibody is also relevant in autoimmune studies, as anti-PDSS2 autoantibodies have been investigated in conditions like systemic sclerosis, though their pathogenic significance remains unclear.

Overall, PDSS2 antibodies are essential for advancing understanding of mitochondrial diseases, CoQ10 biosynthesis, and potential therapeutic interventions.