Rb1051-020809-WS

A synthetic peptide from aa region 160-210 of mouse CNGB3 conjugated to blue carrier protein was used as the antigen.

CNGB3, Cyclic nucleotide-gated cation channel beta-3, Cyclic nucleotide-gated channel beta-3, Cone photoreceptor cGMP-gated channel subunit beta, Cyclic nucleotide-gated cation channel modulatory subunit, Cng6, Cyclic nucleotide-gated channel subunit CNG6

FUNCTION: Visual signal transduction is mediated by a G-protein coupled cascade using cGMP as second messenger. This protein can be activated by cGMP which leads to an opening of the cation channel and thereby causing a depolarization of rod photoreceptors. Induced a flickering channel gating, weakened the outward rectification in the presence of extracellular calcium, increased sensitivity for L-cis diltiazem and enhanced the cAMP efficiency of the channel when coexpressed with CNGA3. Essential for the generation of light-evoked electrical responses in the red-, green- and blue sensitive cones.
Defects in CNGB3 are the cause of Stargardt disease type 1 (STGD1). STGD is one of the most frequent causes of macular degeneration in childhood. It is characterized by macular dystrophy with juvenile-onset, rapidly progressive course, alterations of the peripheral retina, and subretinal deposition of lipofuscin-like material. STGD1 inheritance is autosomal recessive. Defects in CNGB3 are the cause of achromatopsia type 3 (ACHM3); also known as Pingelapese blindness. ACHM3 is a congenital complete achromatopsia and is distinct from total colorblindness mainly because of the consistent concurrence of severe myopia.
Subcellular location: Membrane; Multi-pass membrane protein
Tissue specificity: Expressed specifically in the retina.

Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

12 months after reconstitution

This item will be shipped to you at ambient temperature in a lyophilised form.

1. Gerstner A, et al. J. Neurosci. 20:1324-1332(2000)

For research use only

100 µl

Unconjugated antibody

NZ white rabbit

Whole serum

Polyclonal

Polyclonal, whole serum

IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specific for CNGB3.

Mouse. Other species not yet tested.

Lyophilised

Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.

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