SIL1抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-3: Hela, K562 and HepG2 cell lysates, Primary antibody: P10535(SIL1 Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 10 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P10535(SIL1 Antibody) at dilution 1/130. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P10535(SIL1 Antibody) at dilution 1/130. (Original magnification: ×200)    

  
  

SIL1 antibody targets the SIL1 protein, encoded by the *SIL1* gene, which functions as a nucleotide exchange factor for the endoplasmic reticulum (ER) chaperone BiP (GRP78). This interaction is critical for maintaining protein folding homeostasis in the ER. Mutations in *SIL1* are linked to Marinesco-Sjögren syndrome (MSS), a rare autosomal recessive disorder characterized by cerebellar ataxia, congenital cataracts, and progressive muscle weakness. SIL1 facilitates the release of ADP from BiP, enabling its ATP-bound active state, which is essential for binding and refolding misfolded proteins. Dysfunctional SIL1 disrupts ER stress responses, leading to accumulation of misfolded proteins and cellular apoptosis.

SIL1 antibodies are widely used in research to study protein quality control mechanisms, ER stress-related pathologies, and MSS pathogenesis. They enable detection of SIL1 expression via techniques like Western blotting, immunohistochemistry, and immunofluorescence. Studies using these antibodies have revealed reduced SIL1 levels in MSS patient cells and animal models, highlighting its role in neuronal and muscular health. Additionally, SIL1 antibodies contribute to exploring broader connections between ER dysfunction and neurodegenerative diseases, cancer, or metabolic disorders. Their application aids in elucidating molecular pathways of proteostasis and evaluating potential therapeutic strategies targeting ER stress.