The product of CLCN7 belongs to the CLC chloride channel family of proteins.
Chloride channels play important roles in the plasma membrane and in intracellular organelles.
CLCN7 encodes chloride channel 7.
Defects in CLCN7 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.
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000ELISA: 1:40,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:30ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity:Recognizes endogenous levels of human CLC-7. Species Crossreactivity: mouse, rat
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu Mo Rt
Immunogen:Synthetic peptide corresponding to aa10-90 of human CLC-7 at N-terminal.