CLCN7 Antibody

Catalog Number:

46511

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot:P51798
NCBI Gene ID:1186
NCBI Protein:BC012737

Form of Antibody:

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

Storage/Stability:

Immunogen:

Synthetic protein corresponding to residues near the C terminal of human CLCN7

Purification:

Antigen affinity purification

Specificity/Sensitivity:

The antibody detects endogenous levels of total CLCN7 protein.

Applications:

WB

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.

References:

appl_detail:

Western blotting: 1:200-1:1000
Immunohistochemistry:

Datasheet PDF