CFTR抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P10749(CFTR Antibody) at dilution 1/10. (Original magnification: ×200)    

  
  

The cystic fibrosis transmembrane conductance regulator (CFTR) antibody is a critical tool in studying CFTR, a chloride channel essential for maintaining fluid balance in epithelial tissues. Mutations in the CFTR gene cause cystic fibrosis (CF), a life-limiting genetic disorder characterized by thick mucus accumulation in organs. Researchers use CFTR antibodies to detect, localize, and quantify CFTR protein expression in cells and tissues, aiding in understanding CF pathogenesis, mutation-specific defects (e.g., F508del), and therapeutic responses. These antibodies target specific CFTR domains (e.g., NBD1. R domain) to assess protein folding, trafficking, and function.

In clinical research, CFTR antibodies help evaluate CFTR modulators (e.g., ivacaftor, lumacaftor) that rescue mutant protein function. They are also used in diagnostics to confirm CFTR expression loss in patient-derived samples. Beyond CF, CFTR dysfunction is linked to conditions like chronic pancreatitis and COPD, expanding the antibody’s relevance.

Autoantibodies against CFTR have been reported in autoimmune disorders (e.g., Sjögren’s syndrome), where they may exacerbate exocrine gland dysfunction. However, their clinical significance remains under investigation. Commercial CFTR antibodies vary in specificity, requiring validation to avoid cross-reactivity issues. Overall, CFTR antibodies bridge basic research, drug development, and clinical applications, advancing precision medicine for CF and related diseases.