Calsequestrin1抗体;Calsequestrin1 Antibody—艾普蒂 新品

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     Western blot analysis of Calsequestrin 1 expression in Human skeletal muscle lysate.    

  
  

Calsequestrin 1 (CASQ1) is a calcium-binding protein predominantly localized in the sarcoplasmic reticulum (SR) of skeletal muscle, where it plays a critical role in regulating calcium storage and release during muscle contraction and relaxation. As a high-capacity, low-affinity calcium buffer, CASQ1 aggregates into polymeric matrices that sequester large amounts of calcium ions, ensuring efficient excitation-contraction coupling. Antibodies targeting CASQ1 are essential tools for studying its expression, distribution, and function in both physiological and pathological contexts.

CASQ1 antibodies are widely used in techniques like immunohistochemistry, Western blotting, and immunofluorescence to investigate skeletal muscle development, calcium homeostasis, and diseases such as myopathies or malignant hyperthermia. These antibodies help identify CASQ1 mutations or deficiencies linked to muscle dysfunction. Additionally, they aid in distinguishing CASQ1 from its cardiac isoform, CASQ2. which has distinct tissue specificity and disease associations.

Research involving CASQ1 antibodies has also shed light on its interactions with other SR proteins, like junctophilin and triadin, which form the calcium release complex. Dysregulation of CASQ1 is implicated in vacuolar aggregate myopathy and other neuromuscular disorders, making these antibodies valuable for diagnostic and mechanistic studies. Overall, CASQ1 antibodies serve as crucial reagents for advancing our understanding of muscle biology and calcium signaling pathologies.