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货号： bs-20560R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-20560R-AF555
- 英文名称
- Anti-CNGB3/AF555
- 中文名称
- AF555标记的环核苷酸门控阳离子通道蛋白β3/CNG-β3抗体
- 别 名
- ACHM1; ACHM3; Achromatopsia (rod monochromacy) 3; CNG channel beta-3; CNGB3; CNGB-3_HUMAN; Cone photoreceptor cGMP-gated cation channel beta-subunit; Cone photoreceptor cGMP-gated channel subunit beta; Cyclic nucleotide gated channel beta 3; Cyclic nucleotide-gated cation channel beta-3; Cyclic nucleotide-gated cation channel modulatory subunit; Cyclic nucleotide-gated channel beta-3; RMCH; RMCH1.
- 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 通道蛋白 细胞膜受体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,
- 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
- 92kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CNGB3
- 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:
Cyclic nucleotide-gated (CNG) cation channels are heteromeric complexes made up of principal alpha and modulatory beta subunits. The alpha subunits consist of CNG1-3 and form functional cation channels by themselves. The beta subunits consist of CNG4-6 and, unlike the alpha subunits, do not form functional channels, but rather modify the properties of channels. formed by CNG1-3. CNG channels are essential components of olfactory and visual transduction. CNG proteins are present in cone and rod photoreceptors and in the pineal gland, and they contribute to modulating arterial blood pressure. CNG6, also designated cyclic-nucleotide-gated cation channel beta 3 (CNG-beta 3), is an integral membrane protein that can form a heterooligomeric complex with CNG-3. CNG-beta 3 is activated by cGMP and this activation leads to the depolarization of rod photoreceptors as a result of cation channel being opened. CNG-beta 3 is expressed in a small group of retinal photoreceptor cells and in testis. Mutations in the gene encoding for CNG-beta 3, can cause achromatopsia, an autosomal recessively inherited disease characterized by low visual acuity, photophobia, a lack of color discrimination, and nystagmus.
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 (By similarity). Essential for the generation of light-evoked electrical responses in the red-, green- and blue sensitive cones.
Subunit:
Heterooligomeric complex with CNGA3.
Subcellular Location:
Membrane; Multi-pass membrane protein.
Tissue Specificity:
Expressed specifically in the retina.
DISEASE:
Defects in CNGB3 are the cause of Stargardt disease type 1 (STGD1) [MIM:248200]. 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) [MIM:262300]; 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.
Similarity:
Belongs to the cyclic nucleotide-gated cation channel (TC 1.A.1.5) family.
CNGB3 subfamily.
Contains 1 cyclic nucleotide-binding domain.
Database links:Entrez Gene: 54714 Human
Omim: 605080 Human
SwissProt: Q9NQW8 Human
Unigene: 154433 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.