磷酸二酯酶6β抗体
规格：1尘驳/1尘濒
英文名： PDE6B
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;
分子量： 66kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PDE6B
交叉反应：Human, Mouse, Rat,
细胞定位：细胞膜
磷酸二酯酶6β抗体产物介绍：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磷酸二酯酶6β抗体 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磷酸二酯酶6β抗体 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.
磷酸二酯酶6β抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid