CLCN7 Polyclonal Antibody
规格: | / 20μL / 60μL / 120μL / 200μL |
价格: | / ¥420 / ¥1010 / ¥1640 / ¥2365 |
货号:E-AB-52548
宿主: Rabbit
反应性: H,M,R
应用: IHC
Overview
Synonyms | Chloride channel protein 7,CLC 7,ClC-7,ClC7,CLCN7,CLCN7,FLJ26686,FLJ39644,FLJ46423,H(+)/Cl(-) exchange transporter 7,OPTA2,OPTB4,CLCN7抗体 |
Swissprot | P51798 |
Source | Rabbit |
Reactivity | Human,Mouse,Rat |
Immunogen | Fusion protein of human CLCN7 |
Application | IHC(Detection kit: E-IR-R213) |
Recommended dilution | IHC,,1:50-1:100 |
Concentration | 1.56mg/mL |
Clonality | Polyclonal |
Properties
Cellular localization | Lysosome membrane. |
Isotype | IgG |
Purification | Affinity purification |
Conjugation | Unconjugated |
Storage instructions | Store at -20℃. Avoid freeze / thaw cycles. |
Storage buffer | PBS with 0.05% NaN3 and 40% Glycerol,pH7.4 |
Background | 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. |