The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 110 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport.
Predominantly expressed in skeletal muscles.
Defects in CLCN1 are the cause of Thomsen disease (THD) [MIM:160800]; also known as autosomal dominant myotonia congenita (MCD). THD is characterized by skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability. A variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset and absence of muscle hypo- and hypertrophy. Defects in CLCN1 are the cause of autosomal recessive myotonia congenita (MCR) [MIM:255700]; also known as Becker disease.
Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily. Contains 2 CBS domains.
Predicted band size : 110 kDa Western blot analysis of Mouse muscle membrane fragment labeling CLCN1 with ab110985 at 1:1000. A HRP-conjugated anti-rabbit IgG (1:2000) was used as the secondary antibody. Lane 1 - Mouse muscle membrane fraction. Lane 3 - Mouse brain membrane fraction. Lane 5 - Mouse muscle cytosolic fraction. Lane 6 - Mouse brain cytosolic fraction. Lane 2 and 4 empty.
Western blot - CLCN1 antibody (ab110985)
Anti-CLCN1 antibody (ab110985) at 1 µg/ml + Rat brain lysate at 10 µg