Rabbit PDE6B Polyclonal Antibody | anti-PDE6B antibody

Anti-PDE6B Antibody

Cat. Num. AAA416146

• Reactivity: Human. Predicted: Mouse, Rat. Not yet tested in other species.
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity purified
• Synonyms: PDE6B; Polyclonal Antibody; Anti-PDE6B Antibody; anti-PDE6B antibody

• Host: Rabbit
• Reactivity: Human. Predicted: Mouse, Rat. Not yet tested in other species.
• Clonality: Polyclonal
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 48

• Applicable Applications for anti-PDE6B antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: ELISA titer: 1:20,000-1:80,000; Western blot analysis: 1:500-1:1,000; Immunohistochemistry: 1:100-1:500; Optimal dilutions/concentrations should be determined by the end user
• Storage Buffer: PBS, pH 7.4 with 0.02% Sodium Azide
• Preparation and Storage: This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Immunohistochemistry (IHC)

Related Product Information for anti-PDE6B antibody

Photon absorption triggers a signaling cascade in rod photoreceptors that activates cGMP phosphodiesterase (PDE), resulting in the rapid hydrolysis of cGMP, closure of cGMP-gated cation channels, and hyperpolarization of the cell. PDE is a peripheral membrane heterotrimeric enzyme made up of alpha, beta, and gamma subunits. This gene encodes the beta subunit. Mutations in this gene result in retinitis pigmentosa and autosomal dominant congenital stationary night blindness. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2009]

References

(1) Booij,J.C., Bakker,A., Kulumbetova,J., et al. Simultaneous mutation detection in 90 retinal disease genes in multiple patients using a custom-designed 300-kb retinal resequencing chip. Ophthalmology 118 (1), 160-167 (2011)

NCBI and Uniprot Product Information

• NCBI GI #: 456368294
• Molecular Weight: 66,494 Da
• NCBI Official Full Name: PDE6B, partial
• UniProt Protein Name: Rod cGMP-specific 3',5'-cyclic phosphodiesterase subunit beta
• Protein Family: Rod cGMP-specific 3',5'-cyclic phosphodiesterase
• UniProt Gene Name: PDE6B
• UniProt Entry Name: PDE6B_HUMAN

Uniprot Description

PDE6B: This protein participates in processes of transmission and amplification of the visual signal. Necessary for the formation of a functional phosphodiesterase holoenzyme. Defects in PDE6B are the cause of retinitis pigmentosa type 40 (RP40). RP40 is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Defects in PDE6B are a cause of congenital stationary night blindness autosomal dominant type 2 (CSNBAD2); also known as congenital stationary night blindness Rambusch type. Congenital stationary night blindness is a non-progressive retinal disorder characterized by impaired night vision. Belongs to the cyclic nucleotide phosphodiesterase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Phosphodiesterase; EC 3.1.4.35

Chromosomal Location of Human Ortholog: 4p16.3

Cellular Component: plasma membrane

Molecular Function: 3',5'-cyclic-GMP phosphodiesterase activity; metal ion binding

Biological Process: rhodopsin mediated signaling; phototransduction, visible light; regulation of rhodopsin mediated signaling; cytosolic calcium ion homeostasis; visual perception; retina development in camera-type eye; GMP metabolic process

Disease: Night Blindness, Congenital Stationary, Autosomal Dominant 2; Retinitis Pigmentosa 40