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Rabbit Anti-PDE6B/AF647 Conjugated antibody (bs-12585R-AF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-12585R-AF647
英文名称 Anti-PDE6B/AF647
中文名称 AF647标记的磷酸二酯酶6β抗体
别    名 5''-cyclic phosphodiesterase subunit beta; Congenital stationary night blindness 3 autosomal dominant; CSNB 3; CSNB3; CSNBAD2; GMP PDE beta; GMP-PDE beta; PDE 6 beta; PDE 6B; PDE6B; PDE6B_HUMAN; PDEB; Phosphodiesterase 6B; Phosphodiesterase 6B cGMP specific rod beta; Rd 1; Rd1; Rod cGMP phosphodiesterase beta subunit; Rod cGMP specific 3' 5' cyclic phosphodiesterase beta subunit; Rod cGMP-specific 3''; RP40.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 66kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PDE6B
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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]

Function:
This protein participates in processes of transmission and amplification of the visual signal. Necessary for the formation of a functional phosphodiesterase holoenzyme.

Subcellular Location:
Membrane.

DISEASE:
Defects in PDE6B are the cause of retinitis pigmentosa type 40 (RP40) [MIM:613801]. 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) [MIM:163500]; also known as congenital stationary night blindness Rambusch type. Congenital stationary night blindness is a non-progressive retinal disorder characterized by impaired night vision.

Similarity:
Belongs to the cyclic nucleotide phosphodiesterase family.
Contains 2 GAF domains.

Database links:

Entrez Gene: 281974 Cow

Entrez Gene: 399653 Dog

Entrez Gene: 5158 Human

Entrez Gene: 18587 Mouse

Entrez Gene: 289878 Rat

Omim: 180072 Human

SwissProt: P23439 Cow

SwissProt: P33726 Dog

SwissProt: P35913 Human

SwissProt: P23440 Mouse

Unigene: 623810 Human

Unigene: 654544 Human

Unigene: 1372 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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