Catalog Number:
E20-70961Amount:
100ulMolecular Weight:
88679DaApplications:
WB;IHC-p;IF(paraffin section);ELISAReactivity:
Human;MousePurification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.Immunogen:
The antiserum was produced against synthesized peptide derived from human CLCN7. AA range:10-59Source:
Polyclonal, Rabbit,IgG
Gene Name:
CLCN7Dilution:
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.Protein Name:
H(+)/Cl(-) exchange transporter 7Human Gene Id:
1186Human Swiss Prot No:
P51798Mouse Gene Id:
26373Mouse Swiss Prot No:
O70496Rat Gene Id:
29233Rat Swiss Prot No:
P51799Specificity:
CLC-7 Polyclonal Antibody detects endogenous levels of CLC-7 protein.Formulation:
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.Concentration:
1 mg/mlStorage Stability:
-20°C/1 yearOther Name:
CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7Observed Band(KD):
90Background:
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],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 ioSubcellular Location:
Lysosome membrane ; Multi-pass membrane protein .Expression:
Brain and kidney.Products Images
Catalog Number:
E011009-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
145$
Catalog Number:
E011009-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
225$
Catalog Number:
E011013-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
145$
Catalog Number:
E011013-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
225$
Catalog Number:
E011020-1Amount:
50μg/50μlReactivity:
HumanApplications:
IF:1:100~1:200Swiss-Prot No.:
Q04864Price:
145$