| 產品編號 | bs-20560R |
| 英文名稱 | Rabbit Anti-CNGB3 antibody |
| 中文名稱 | 環核苷酸門控陽離子通道蛋白β3/CNG-β3抗體 |
| 別 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. |
| 研究領域 | 細胞生物 神經生物學 通道蛋白 細胞膜受體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | (predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Chicken,Dog) |
| 產品應用 | WB=1:500-2000,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 92kDa |
| 細胞定位 | 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CNGB3: 301-400/809 <Extracellular> |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產品介紹 |
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. SWISS: Q9NQW8 Gene ID: 54714 Database links: Entrez Gene: 54714 Human Omim: 605080 Human SwissProt: Q9NQW8 Human Unigene: 154433 Human |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
