FITC标记的突触受体相关蛋白43抗体
产品名称： FITC标记的突触受体相关蛋白43抗体
英文名称： Anti-Rapsyn/FITC
产品编号： HZ-19664R-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-Rapsyn/FITC Conjugated antibody
FITC标记的突触受体相关蛋白43抗体
| 英文名称 | Anti-Rapsyn/FITC |
| 中文名称 | FITC标记的突触受体相关蛋白43抗体 |
| 别 名 | 43 kD receptor associated protein of the synapse; 43 kda postsynaptic protein; 43 kDa receptor-associated protein of the synapse; Acetylcholine receptor associated 43 kda protein; Acetylcholine receptor-associated 43 kDa protein; CMS1D; CMS1E; MGC3597; RAPSN; RAPSN_HUMAN; RAPsyn; Receptor associated protein of the synapse 43kD; Receptor associated protein of the synapse; RING finger protein 205; RNF 205; RNF205. |
| 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 免疫学 神经生物学 细胞骨架 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Xenopus laevis |
| 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 | 38kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Rapsyn |
| 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: This gene encodes a member of a family of proteins that are receptor associated proteins of the synapse. The encoded protein contains a conserved cAMP-dependent protein kinase phosphorylation site, and plays a critical role in clustering and anchoring nicotinic acetylcholine receptors at synaptic sites by linking the receptors to the underlying postsynaptic cytoskeleton, possibly by direct association with actin or spectrin. Mutations in this gene may play a role in postsynaptic congenital myasthenic syndromes. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Apr 2011] Function: Thought to play some role in anchoring or stabilizing the nicotinic acetylcholine receptor at synaptic sites. It may link the receptor to the underlying postsynaptic cytoskeleton, possibly by direct association with actin or spectrin. Subcellular Location: Cell membrane. Cell junction > synapse > postsynaptic cell membrane. Cytoplasm > cytoskeleton. Cytoplasmic surface of postsynaptic membranes. DISEASE: Defects in RAPSN are a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (ACHRDCMS) [MIM:608931]. ACHRDCMS is a post-synaptic congenital myasthenic syndrome. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie 'slow-channel myasthenic syndromes' (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in 'fast-channel syndromes' (FCCMS). ACHRDCMS is the third disorder associated with postsynaptic CMS which could result from mutations in the proteins forming the muscle AChR. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance. Defects in RAPSN are the cause of fetal akinesia deformation sequence (FADS) [MIM:208150]; also known as Pena-Shokeir syndrome type 1 or fetal akinesia sequence or arthrogryposis multiplex congenita with pulmonary hypoplasia. FADS is a rare condition characterized by decreased intrauterine fetal movement, congenital limb contractures, pulmonary hypoplasia, polyhydramnios and craniofacial abnormalities. Similarity: Belongs to the RAPsyn family. Contains 1 RING-type zinc finger. Contains 7 TPR repeats. Database links: Entrez Gene: 5913 Human Entrez Gene: 19400 Mouse Entrez Gene: 362161 Rat Omim: 601592 Human SwissProt: Q13702 Human SwissProt: P12672 Mouse Unigene: 81218 Human Unigene: 1272 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
这个基因编码一个蛋白质家族的成员，这些蛋白质是突触的受体相关蛋白。该编码蛋白包含一个保守的cAMP依赖性蛋白激酶磷酸化位点，并且通过将受体连接到潜在的突触后细胞骨架上，可能通过直接结合，在突触部位聚集和锚定烟碱乙酰胆碱受体中发挥关键作用。肌动蛋白或血影蛋白。该基因的突变可能在突触后先天性肌无力综合征中发挥作用。另一种编码多个异构体的剪接转录变体已被观察到。