CLC-7 rabbit pAb

CLC-7 rabbit pAb

• Description: Chloride voltage-gated channel 7 (CLCN7) Homo sapiens The product of this gene belongs to the CLC chloride channel family of proteins. Chloride channels play important roles in the plasma membrane and in intracellular organelles. This gene encodes chloride channel 7. Defects in this gene are the cause of osteopetrosis autosomal recessive type 4 (OPTB4), also called infantile malignant osteopetrosis type 2 as well as the cause of autosomal dominant osteopetrosis type 2 (OPTA2), also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood. [provided by RefSeq, Jul 2008],
• Synonyms: CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7
• Gene ID: 1186
• UniProt: P51798
• Cellular Locus: Lysosome membrane ; Multi-pass membrane protein.
• Host: Rabbit
• Species Reactivity: Human, Mouse, Rat
• Immunogen: The antiserum was produced against synthesized peptide derived from human CLCN7. AA range:10-59
• Clonality: Polyclonal
• Validated Applications: WB, ELISA
• Stability: 1 year
• Concentration: 1 mg/mL
• Dilution: Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
• Molecular Weight: 90kD
• Storage Conditions: PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.