概述

  • 产品名称
  • 描述
    兔多克隆抗体to MUSK
  • 宿主
    Rabbit
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
    不与反应: Rat
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 300 - 400 of Human MUSK.

    参阅Abcam的专有抗源政策

  • 阳性对照
    • This antibody gave a positive signal in Human and Mouse Skeletal Muscle Tissue Lysates.

性能

应用

Our Abpromise guarantee covers the use of ab92950 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
WB Use a concentration of 1 µg/ml. Detects a band of approximately 105 kDa (predicted molecular weight: 97 kDa).

靶标

  • 功能
    Receptor tyrosine kinase that is a key mediator of agrin's action and is involved in neuromuscular junction (NMJ) organization.
  • 疾病相关
    Defects in MUSK is a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (CMS-ACHRD) [MIM:608931]. A post-synaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance. Note=MUSK mutations lead to decreased agrin-dependent AChR aggregation, a critical step in the formation of the neuromuscular junction.
  • 序列相似性
    Belongs to the protein kinase superfamily. Tyr protein kinase family.
    Contains 1 FZ (frizzled) domain.
    Contains 3 Ig-like C2-type (immunoglobulin-like) domains.
    Contains 1 protein kinase domain.
  • 翻译后修饰
    Ubiquitinated by PDZRN3. Ubiquitination promotes endocytosis and lysosomal degradation.
  • 细胞定位
    Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • CMS9 antibody
    • FADS antibody
    • MDK 4 antibody
    • MDK4 antibody
    • MGC126323 antibody
    • MGC126324 antibody
    • Muscle antibody
    • Muscle associated receptor tyrosine kinase antibody
    • Muscle skeletal receptor tyrosine kinase antibody
    • Muscle skeletal receptor tyrosine protein kinase antibody
    • Muscle specific kinase receptor antibody
    • Muscle specific tyrosine kinase receptor antibody
    • Muscle specific tyrosine protein kinase receptor antibody
    • Muscle-specific kinase receptor antibody
    • Muscle-specific tyrosine-protein kinase receptor antibody
    • MuSK antibody
    • MUSK_HUMAN antibody
    • Neural fold somite kinase 1 antibody
    • Neural fold somite kinase 2 antibody
    • Neural fold somite kinase 3 antibody
    • Neural fold somite kinase1 antibody
    • Neural fold somite kinase2 antibody
    • Neural fold somite kinase3 antibody
    • Nsk 1 antibody
    • Nsk 2 antibody
    • Nsk 3 antibody
    • Nsk1 antibody
    • Nsk2 antibody
    • Nsk3 antibody
    • Receptor tyrosine kinase MuSK antibody
    • Skeletal muscle receptor tyrosine kinase antibody
    • skeletal receptor tyrosine-protein kinase antibody
    see all

图片

  • All lanes : Anti-MUSK antibody (ab92950) at 1 µg/ml

    Lane 1 : Human skeletal muscle tissue lysate - total protein (ab29330)
    Lane 2 : Skeletal Muscle (Mouse) Tissue Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 97 kDa
    Observed band size: 105 kDa
    why is the actual band size different from the predicted?
    Additional bands at: 65 kDa, 80 kDa. We are unsure as to the identity of these extra bands.


    Exposure time: 2 minutes


    MUSK protein sequence contains two potential glycosylation sites (SwissProt Data), which may explain the migration of the band at a higher molecular weight than predicted.

实验方案

文献

This product has been referenced in:
  • Logan CV  et al. Congenital Myasthenic Syndrome Type 19 Is Caused by Mutations in COL13A1, Encoding the Atypical Non-fibrillar Collagen Type XIII a1 Chain. Am J Hum Genet 97:878-85 (2015). Read more (PubMed: 26626625) »
See 1 Publication for this product

客户评价及客户问答

1-2 of 2 Abreviews or Q&A

Abcam has not validated the combination of species/application used in this Abreview.
Application
Western blot
Sample
Rat Cell lysate - whole cell (HEK293 cells transfected with rat MuSK)
Loading amount
30 µg
Specification
HEK293 cells transfected with rat MuSK
Gel Running Conditions
Reduced Denaturing (7,5% polyacrylamide)
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C

Abcam user community

Verified customer

提交于 Sep 06 2012

Answer

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