Anti-CLCN1 Antibody

The skeletal muscle chloride channel ClC-1, encoded by the CLCN1 gene, plays a critical role in stabilizing the resting membrane potential of muscle fibers and repolarizing the sarcolemma after action potentials. This unconjugated rabbit polyclonal reagent from Antibodies.com is raised against CLCN1 and validated for western blot and immunohistochemistry workflows. It recognizes the target in both human and mouse samples, supporting cross-species studies of inherited muscle disorders. CLCN1 loss-of-function mutations are the molecular basis of myotonia congenita, so the antibody is widely used to investigate channel expression, sarcolemmal localization, and trafficking defects in patient-derived myotubes and relevant animal models. The preparation is supplied as 100 µl of affinity-purified IgG at 1 mg/ml in PBS containing 0.1% sodium azide and 50% glycerol as a cryoprotectant. Aliquot upon receipt and store at -20 °C, avoiding repeated freeze-thaw cycles to preserve binding activity. The polyclonal format provides recognition of multiple epitopes across the channel protein, which is useful when probing for mutant or truncated variants of ClC-1 in skeletal muscle lysates and tissue sections.