概述

  • 产品名称Anti-Rapsyn抗体[1234]
    参阅全部 Rapsyn 一抗
  • 描述
    小鼠单克隆抗体[1234] to Rapsyn
  • 经测试应用适用于: IP, ICC, IHC-Fr, WB, ICC/IFmore details
  • 种属反应性
    与反应: Mouse, Rat, Chicken, Human, Xenopus laevis, Fish, Amphibians, Xenopus tropicalis
  • 免疫原

    Other Immunogen Type corresponding to Rapsyn. Whole purified rapsyn from Torpedo californica electric organ postsynaptic membrane.

  • 阳性对照
    • Torpedo californica electrocyte cell extracts.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液Preservative: 0.05% Sodium azide
    Constituents: 99% PBS, 0.1% BSA
  • Concentration information loading...
  • 纯度Protein G purified
  • 克隆单克隆
  • 克隆编号1234
  • 同种型IgG1
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab11423 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IP Use at an assay dependent concentration.
ICC Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
WB Use a concentration of 0.5 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 46 kDa).

Detects a band of approximately 48 kDa, representing rapsyn from Torpedo californica electrocyte cell extracts.

ICC/IF Use a concentration of 2 µg/ml.

靶标

  • 功能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.
  • 疾病相关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.
  • 序列相似性Belongs to the RAPsyn family.
    Contains 1 RING-type zinc finger.
    Contains 7 TPR repeats.
  • 结构域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.
  • 细胞定位Cell membrane. Cell junction > synapse > postsynaptic cell membrane. Cytoplasm > cytoskeleton. Cytoplasmic surface of postsynaptic membranes.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 43 kD receptor associated protein of the synapse antibody
    • 43 kDa postsynaptic protein antibody
    • 43 kDa receptor-associated protein of the synapse antibody
    • Acetylcholine receptor associated 43 kda protein antibody
    • Acetylcholine receptor-associated 43 kDa protein antibody
    • CMS1D antibody
    • CMS1E antibody
    • MGC3597 antibody
    • RAPSN antibody
    • RAPSN_HUMAN antibody
    • RAPsyn antibody
    • Receptor associated protein of the synapse 43kD antibody
    • Receptor associated protein of the synapse antibody
    • RING finger protein 205 antibody
    • RNF 205 antibody
    • RNF205 antibody
    see all

Anti-Rapsyn antibody [1234] (ab11423)参考文献

This product has been referenced in:

See all 3 Publications for this product

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