Produced in rabbits immunized with E. coli-derived Human CLCN2 fragment, and purified by antigen affinity chromatography.
Source
Polyclonal Rabbit IgG
Purification
Protein A & Antigen Affinity
Formulation
PBS, pH7.0 with 0.03% Proclin300
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Storage
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Avoid repeated freeze-thaw cycles.
Immunofluorescence staining of CLCN2 in CACO2 cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human CLCN2 polyclonal antibody (dilution ratio 1:200) at 4℃ overnight. Then cells were stained with the Alexa Fluor®488-conjugated Goat Anti-rabbit IgG secondary antibody (green) and counterstained with DAPI (blue).Positive staining was localized to Cytoplasm.
CLCN2 encodes a voltage-gated chloride channel protein 2 (ClC-2) expressed in adrenal glomerulosa that opens at hyperpolarized membrane potentials. Channel opening depolarizes glomerulosa cells and induces expression of aldosterone synthase, the rate-limiting enzyme for aldosterone biosynthesis. Mutant channels show gain of function, with higher open probabilities at the glomerulosa resting potential. CLCN2 mutation is associated with familial hyperaldosteronism II (FH II) and CLCN2-related leukoencephalopathy (CC2L).
References
Scholl UI, et al. (2018) Clcn2 chloride channel mutations in familial hyperaldosteronism type ii. Nat Genet 50 (3): 349-354.