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Product Name
CLCN7 Rabbit pAb
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Gene ID
1186
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SwissProt ID
P51798
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Gene Name
CLCN7
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Alternative Names
chloride voltage-gated channel 7; HOD; CLC7; CLC-7; OPTA2; OPTB4; PPP1R63
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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.
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Research Field
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Product Categories
Primary antibody
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Host
Rabbit
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Reactivity
Human,Mouse,Rat
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Application
WB,IHC-P
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Dilution Ratio
WB: 1/500-1/1000 IHC: 1/50-1/100
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Molecular Weight
Calculated MW: 89 kDa; Observed MW: 89 kDa
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Clonality
Polyclonal Antibody
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Clonality No.
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Isotype
IgG
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Immunogen
Fusion protein of human CLCN7
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Purification
Affinity Purified
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Conjugation
Unconjugated
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Modification
Unmodified
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Form
Liquid
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Buffer System
pH 7.4 PBS, 0.05% NaN3, 40% Glycerol
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Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.