CLC-7 Rabbit Polyclonal Antibody
Konjugasyon: Unconjugated
Rabbit polyclonal Antibody
Uygulama
Reaktivite
Human,Mouse,Rat
Gen Adı
CLCN7
Saklama
Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles.
Özet
| Ürün Adı | CLC-7 Rabbit Polyclonal Antibody |
| Açıklama | Rabbit polyclonal Antibody |
| Konak | Rabbit |
| Reaktivite | Human,Mouse,Rat |
| Konjugasyon | Unconjugated |
| Modifikasyon | Unmodified |
| İzotip | IgG |
| Klonalite | Polyclonal |
| Form | Liquid |
| Konsantrasyon | Unconjugated |
| Saklama | Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles. |
| Nakliye | Ice bags. |
| Tampon | Liquid in PBS containing 50% glycerol, 0.5% protective protein and 0.02% New type preservative N. |
| Saflaştırma | Affinity purification |
Antijen Bilgisi
| Gen Adı | CLCN7 |
| Alternatif İsimler | CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7 |
| Gen Kimliği | 1186 |
| SwissProt Kimliği | P51798 |
| İmmünojen | The antiserum was produced against synthesized peptide derived from human CLCN7. AA range:10-59 |
Uygulama
| Uygulama | WB,ELISA |
| Seyreltme Oranı | WB 1:500-1:2000,ELISA 1:20000-1:40000 |
| Moleküler Ağırlık | 90kDa |
Araştırma Alanı
Arka Plan
| 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],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., |
