FITC标记的环核苷酸门控阳离子通道蛋白β3/CNG-β3抗体
产品名称： FITC标记的环核苷酸门控阳离子通道蛋白β3/CNG-β3抗体
英文名称： Anti-CNGB3/FITC
产品编号： HZ-12094R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-CNGB3/FITC Conjugated antibody
FITC标记的环核苷酸门控阳离子通道蛋白β3/CNG-β3抗体
| 英文名称 | Anti-CNGB3/FITC |
| 中文名称 | FITC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, Horse, |
| 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 |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 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 |
环核苷酸门控（CNG）阳离子通道是由主α和调节β亚基组成的异构化复合物。α亚基由CNG1-3组成，并通过自身形成功能阳离子通道。β亚基由CNG4-6组成，与α亚基不同，β亚基不形成功能通道，而是改变通道的性质。由CNG1-3形成。CNG通道是嗅觉和视觉传导的重要组成部分。CNG蛋白存在于锥体和杆状光感受器和松果腺中，它们有助于调节动脉血压。CNG6，也称为环核苷酸门控阳离子通道β3（CNG-β3），是一种完整的膜蛋白，能与CNG-3形成异同工酶复合物。CNG-β3被cGMP激活，这种激活导致杆状光感受器的去极化，这是阳离子通道被打开的结果。CNGβ3在小的视网膜感光细胞和睾丸中表达。编码CNG-β3的基因突变可导致无色盲，这是一种常染色体隐性遗传疾病，特征是视力低下、畏光、缺乏颜色辨别和眼震。