COPA抗体;COPA Antibody—艾普蒂 新品

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     Western blot analysis of COPA expression in MCF7 cell lysate.    

  
  

COPA antibodies are autoantibodies associated with a rare autoimmune disorder known as COPA syndrome, linked to mutations in the *COPA* gene. The *COPA* gene encodes the coatomer protein complex subunit alpha, a component of the COPI vesicular transport system responsible for intracellular protein trafficking between the Golgi apparatus and endoplasmic reticulum. Dysfunctional COPA disrupts proper protein sorting, leading to endoplasmic reticulum stress and abnormal immune activation.

Clinically, COPA syndrome manifests early in life (often childhood) with pulmonary hemorrhage, interstitial lung disease, and autoimmune features like arthritis or kidney involvement. Anti-COPA antibodies are detected in some patients, though their pathogenic role remains unclear. These antibodies are part of a broader autoantibody profile that may include anti-nuclear antibodies (ANA) or rheumatoid factor (RF).

Diagnostically, COPA antibody testing aids in differentiating COPA syndrome from other autoimmune or interstitial lung diseases. However, diagnosis primarily relies on genetic testing for *COPA* mutations, as antibody presence alone is nonspecific. Research continues to explore the interplay between COPA dysfunction, autoimmunity, and targeted therapies to modulate immune responses in affected patients.