Fibrillarin抗体;Fibrillarin Antibody—艾普蒂

+ +

•  

   

     All lanes use the Antibody at 1:2K dilution for 1 hour at room temperature.    

  
  

•  

   

     All lanes use the Antibody at 1:2K dilution for 1 hour at room temperature.    

  
  

•  

   

     Western blot analysis of Fibrillarin expression in HepG2 cell lysate.    

  
  

•  

   

     Immunofluorescent analysis using the Antibody at 1:50 dilution.    

  
  

•  

   

     Immunofluorescent analysis of Hela cells, using Fibrillarin Antibody .    

  
  

•  

   

     Immunohistochemical analysis of paraffin-embedded human testis, using Fibrillarin Antibody.    

  
  

Fibrillarin antibodies are autoimmune antibodies primarily associated with systemic sclerosis (scleroderma) and related disorders. Fibrillarin, a highly conserved nucleolar protein, plays a critical role in ribosomal RNA (rRNA) processing and ribosome assembly. It is a core component of small nucleolar ribonucleoprotein (snoRNP) complexes, facilitating site-specific methylation of pre-rRNA during ribosome biogenesis. Structurally, fibrillarin contains a glycine- and arginine-rich (GAR) domain and a conserved methyltransferase domain, localizing predominantly within the dense fibrillar regions of the nucleolus.

In autoimmune contexts, anti-fibrillarin antibodies (AFAs) target this protein, often yielding a distinctive "clumpy" nucleolar staining pattern in indirect immunofluorescence (IIF) assays. These autoantibodies are detected in approximately 5-10% of systemic sclerosis patients, particularly those with diffuse cutaneous involvement or overlapping connective tissue diseases. Their presence correlates with severe clinical manifestations, including pulmonary hypertension and cardiac complications. AFAs are also linked to murine models of autoimmunity, such as the MRL/lpr lupus-prone mice, supporting their role in disease pathogenesis.

Clinically, AFA testing aids in diagnosing and stratifying autoimmune patients, while research applications include studying nucleolar organization, RNA metabolism, and autoimmune-triggered molecular pathways. Commercial fibrillarin antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate cellular stress responses, cancer biology, and nucleolar dysfunction mechanisms.