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品种反应性 主要应用 宿主 格式 抗体类型 R, M WB M Purified Monoclonal Antibody 描述 产品目录编号 MAB2238 描述 Anti-Rapsyn Antibody, clone1234 Alternate Names - receptor-associated protein of the synapse, 43kD
- 43 kD receptor-associated protein of the synapse
- 43 kDa postsynaptic protein
- Acetylcholine receptor-associated 43 kDa protein
- RING finger protein 205
- receptor-associated protein of the synapse
背景信息 Rapsyn (receptor-associated protein of the synapse) is a peripheral membrane protein that has a close association to acetylcholine receptors (AChRs). Studies have shown that mice with a mutated rapsyn gene were unable to form AChR clusters, resulting in birth from respiratory failure death soon after. Other mutations affecting the activity of rapsyn can lead to the formation of the neuromuscular disease myasthenia gravis. 产品信息 格式 Purified 控制 - C2C12 cell lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-glycine, pH 7.4, 150 mM NaCl and 0.05% sodium azide. 应用 应用 This Anti-Rapsyn Antibody, clone1234 is validated for use in WB for the detection of Rapsyn. 主要应用 - Western Blotting
生物信息 免疫原品种 This antibody is generated from purified rapsyn of Torpedo californica electric organ postsynaptic membrane. 克隆 1234 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 This antibody recognizes Rapsyn. 同种型 IgG1κ 品种反应性 RatMouse Species Reactivity Note Proven to react with mouse and rat. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_033049.2
基因符号 - rapsyn
- CMS1D
- CMS1E
- MGC3597
- RAPsyn
- RNF205
纯化方法 Protein G purfied UniProt编号 - Q13702
UniProt汇总 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; Peripheral membrane protein; Cytoplasmic side. Cell junction › synapse › postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm › cytoskeleton. Note: Cytoplasmic surface of postsynaptic membranes.
DOMAIN: A cysteine-rich region homologous to part of the regulatory domain of protein kinase C may be important in interactions of this protein with the lipid bilayer.
INVOLVEMENT IN DISEASE: Defects in RAPSN are a cause of congenital myasthenic syndrome type 1d (CMS1D) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. 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). CMS1D 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.
SEQUENCE SIMILARITIES: Belongs to the RAPsyn family.
Contains 1 RING-type zinc finger.
Contains 7 TPR repeats.产品使用声明 质量保证 Evaluated by Western Blot in C2C12 cell lysate.
Western Blot Analysis: : 5 µg/mL dilution of this antibody detected Rapsyn on 15 µg of C2C12 cell lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain refrigerated at 2-8°C for 1 year from date of receipt. 包装信息 数量 100 µg
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