Anti-Alpha Skeletal Muscle Actin抗体(ab113417)

概述

  • 产品名称Anti-Alpha Skeletal Muscle Actin抗体
    参阅全部 Alpha Skeletal Muscle Actin 一抗
  • 描述
    兔多克隆抗体to Alpha Skeletal Muscle Actin
  • 特异性ab113417 does not react to smooth muscle actin.
  • 经测试应用适用于: WB, IP, IHC-Pmore details
  • 种属反应性
    与反应: Mouse, Rat, Chicken, Cow, Human
  • 免疫原

    Synthetic peptide derived from N-terminus of Human skeletal muscle Actin. This sequence is identical in Human, Rat, Mouse, Dog, Bovine, Guinea pig, Sheep and Frog origins.

  • 阳性对照
    • Mouse Skeletal Muscle lysate

性能

应用

Our Abpromise guarantee covers the use of ab113417 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 0.1 - 1 µg/ml. Detects a band of approximately 42 kDa (predicted molecular weight: 42 kDa).
IP Use a concentration of 2 - 5 µg/ml.
IHC-P Use a concentration of 2 - 5 µg/ml. Staining of formalin-fixed tissue requires boiling tissue sections in 10 mM Citrate Buffer, pH 6.0 for 10 min followed by cooling at RT for 20 min.

靶标

  • 功能Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
  • 疾病相关Defects in ACTA1 are the cause of nemaline myopathy type 3 (NEM3) [MIM:161800]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. The phenotype at histological level is variable. Some patients present areas devoid of oxidative activity containg (cores) within myofibers. Core lesions are unstructured and poorly circumscribed.
    Defects in ACTA1 are a cause of myopathy congenital with excess of thin myofilaments (MPCETM) [MIM:161800]. A congenital muscular disorder characterized at histological level by areas of sarcoplasm devoid of normal myofibrils and mitochondria, and replaced with dense masses of thin filaments. Central cores, rods, ragged red fibers, and necrosis are absent.
    Defects in ACTA1 are a cause of congenital myopathy with fiber-type disproportion (CFTD) [MIM:255310]; also known as congenital fiber-type disproportion myopathy (CFTDM). CFTD is a genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific and can be found in many different myopathic and neuropathic conditions.
  • 序列相似性Belongs to the actin family.
  • 细胞定位Cytoplasm > cytoskeleton.
  • Information by UniProt
  • 数据库链接
  • 别名
    • a actin antibody
    • ACTA antibody
    • ACTA1 antibody
    • ACTC antibody
    • ACTC1 antibody
    • Actin alpha cardiac muscle antibody
    • Actin, alpha skeletal muscle antibody
    • ACTS_HUMAN antibody
    • Alpha actin 1 antibody
    • Alpha-actin-1 antibody
    • ASMA antibody
    • Cardiac muscle alpha actin 1 antibody
    • MPFD antibody
    • Skeletal muscle alpha actin 1 antibody
    see all

Anti-Alpha Skeletal Muscle Actin antibody 图像

  • Anti-Alpha Skeletal Muscle Actin antibody (ab113417) at 1/500 dilution + Mouse skeletal muscle lysate

    Predicted band size : 42 kDa
  • Skeletal muscle tissue stained with ab113417 at a dilution of 1/200.

Anti-Alpha Skeletal Muscle Actin antibody (ab113417)参考文献

This product has been referenced in:
  • Azakir BA  et al. Proteasome inhibitors increase missense mutated dysferlin in patients with muscular dystrophy. Sci Transl Med 6:250ra112 (2014). Human . Read more (PubMed: 25143362) »

See 1 Publication for this product

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Expiration date: June 15th, 2012
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"