- CITATIONS: 1
|Application ||WB, IHC-P, IF, E|
|Other Accession||P51799, O70496, Q4PKH3, NP_001278.1|
|Predicted||Bovine, Mouse, Rat|
|Calculated MW||88679 Da|
|Antigen Region||692-720 aa|
|Other Names||H(+)/Cl(-) exchange transporter 7, Chloride channel 7 alpha subunit, Chloride channel protein 7, ClC-7, CLCN7|
|Target/Specificity||This CLCN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 692-720 amino acids from the C-terminal region of human CLCN7.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||CLCN7 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.|
|Cellular Location||Lysosome membrane; Multi-pass membrane protein|
|Tissue Location||Brain, testis, muscle and kidney.|
Provided below are standard protocols that you may find useful for product applications.
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.
Furthner, D., et al. Klin Padiatr 222(3):180-183(2010)
Phadke, S.R., et al. Indian J. Med. Res. 131, 508-514 (2010) :
Pangrazio, A., et al. Hum. Mutat. 31 (1), E1071-E1080 (2010) :
Kajiya, H., et al. Pflugers Arch. 458(6):1049-1059(2009)
Mazzolari, E., et al. Am. J. Hematol. 84(8):473-479(2009)
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