ITT0961
ITT0961
- Catalog: ITT0961
- Gene/Protein: CLCN7
- Product Description: Immunotag™ CLC-7 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | CLC-7 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from CLC-7, at AA range: 10-90 |
| Specificity | CLC-7 Polyclonal Antibody detects endogenous levels of CLC-7 protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | CLCN7 |
| Accession No. | P51798 O70496 P51799 |
| Alternate Names | CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7 |
| 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], |
| Protein Expression | Brain,Epithelium,Lens epithelium,Mammary gland,PCR rescued clones,Skin,Test |
| Subcellular Localization | lysosomal membrane,membrane,integral component of membrane,cytoplasmic vesicle, |
| Protein Function | disease:Defects in CLCN7 are a cause of autosomal dominant osteopetrosis type 2 (OPTA2) [MIM:166600]; also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood. It is characterized by sclerosis, predominantly involving the spine, the pelvis, and the skull base.,disease:Defects in CLCN7 are the cause of osteopetrosis autosomal recessive type 4 (OPTB4) [MIM:611490]; also called infantile malignant osteopetrosis type 2. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood.,function:Mediates the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.,miscellaneous:The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The presence of conserved gating glutamate residues is typical for family members that function as antiporters.,similarity:Belongs to the chloride channel (TC 2.A.49) family.,similarity:Contains 2 CBS domains.,tissue specificity:Brain, testis, muscle and kidney., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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