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货号： bs-12585R-Gold 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12585R-Gold
- 英文名称
- Anti-PDE6B/Gold
- 中文名称
- 胶体金标记的磷酸二酯酶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（10nm 15nm 35nm）
- 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
- Human, Mouse, Rat,
- 产品应用
- IEM=1:20-200 IGS=1:20-200 GICA=1:20-200
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
- 0.02M TBS(pH8.2) 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: 281974Cow
Entrez Gene: 399653Dog
Entrez Gene: 5158Human
Entrez Gene: 18587Mouse
Entrez Gene: 289878Rat
Omim: 180072Human
SwissProt: P23439Cow
SwissProt: P33726Dog
SwissProt: P35913Human
SwissProt: P23440Mouse
Unigene: 623810Human
Unigene: 654544Human
Unigene: 1372Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.