ATP7B 抗体;ATP7B Antibody—艾普蒂

+ +

•  

   

     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P12465(ATP7B  Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

•  

   

     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P12465(ATP7B  Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

ATP7B antibody is a crucial tool in studying Wilson disease, a rare autosomal recessive disorder caused by mutations in the ATP7B gene. The ATP7B protein, a copper-transporting P-type ATPase, plays a vital role in copper homeostasis by facilitating its excretion into bile and incorporation into ceruloplasmin. Expressed predominantly in the liver, ATP7B maintains systemic copper balance by trafficking between the trans-Golgi network and cytoplasmic vesicles under varying copper levels. Mutations in ATP7B disrupt copper excretion, leading to toxic copper accumulation in the liver, brain, and other organs, resulting in Wilson disease's clinical manifestations, including liver cirrhosis and neurological deficits.

Antibodies targeting ATP7B are widely used in research and diagnostics to detect protein expression, localization, and functional abnormalities. In research, these antibodies enable techniques like Western blotting, immunohistochemistry, and immunofluorescence to study ATP7B's role in cellular copper regulation and disease mechanisms. Clinically, they assist in confirming Wilson disease diagnoses by identifying reduced or mislocalized ATP7B in liver biopsies. Additionally, ATP7B antibodies contribute to therapeutic development, such as evaluating gene therapy efficacy or small-molecule interventions aimed at restoring copper transport. Their specificity and reliability make them indispensable for advancing understanding and management of copper metabolism disorders.