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.
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, ELISA
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Specificity：Human
Isotype：IgG
Calc Applications Abbrev：E IF IHC WB
Calc Crossreactivity：Hu
Immunogen：CLCN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 699-728 amino acids from the C-terminal region of human CLCN7.
