catalog number :
MBS4154824
products full name :
Anti-CLCN7 Antibody
products short name :
[CLCN7]
other names :
[CLCN7 protein, partial; H(+)/Cl(-) exchange transporter 7; H(+)/Cl(-) exchange transporter 7; chloride voltage-gated channel 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7]
products gene name :
[CLCN7]
other gene names :
[CLCN7; CLCN7; CLC7; CLC-7; OPTA2; OPTB4; PPP1R63; ClC-7]
reactivity :
Human, Mouse, Rat
purity :
Affinity Purification
storage stability :
Store at -20 degree C. Avoid freeze/thaw cycles.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
app notes :
WB: 1:500-1:2000. IHC: 1:50-1:200. IF: 1:50-1:100
other info1 :
Immunogen: Recombinant protein of human CLCN7. Storage Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
products description :
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 ost.
ncbi acc num :
AAI09197.1
ncbi summary :
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]
uniprot summary :
Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.