概述

  • 产品名称Anti-Myotilin抗体
    参阅全部 Myotilin 一抗
  • 描述
    兔多克隆抗体to Myotilin
  • 经测试应用适用于: IHC-P, WBmore details
  • 种属反应性
    与反应: Rat
    预测可用于: Mouse, Cow, Human
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Myotilin.

    (Peptide available as ab74353.)

  • 阳性对照
    • This antibody gave a positive signal in Rat Skeletal Muscle Tissue Lysate.

性能

应用

Our Abpromise guarantee covers the use of ab68915 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
IHC-P Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 56 kDa (predicted molecular weight: 56 kDa).

靶标

  • 功能Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells.
  • 组织特异性Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.
  • 疾病相关Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A) [MIM:159000]. LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line.
    Defects in MYOT are the cause of myopathy myofibrillar myotylin-related (MFM-MYOT) [MIM:609200]. A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits.
    Defects in MYOT are the cause of spheroid body myopathy (SBM) [MIM:182920]. SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers.
  • 序列相似性Belongs to the myotilin/palladin family.
    Contains 2 Ig-like C2-type (immunoglobulin-like) domains.
  • 细胞定位Cell membrane > sarcolemma. Cytoplasm > cytoskeleton. Cytoplasm > myofibril > sarcomere > Z line. Sarcomeric, also localized to the sarcolemma. Colocalizes with MYOZ1 at the Z-lines in skeletal muscle.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 57 kDa cytoskeletal protein antibody
    • LGMD 1 antibody
    • LGMD1 antibody
    • Myofibrillar titin like Ig domains protein antibody
    • Myofibrillar titin-like Ig domains protein antibody
    • Myot antibody
    • MYOTI_HUMAN antibody
    • Myotilin antibody
    • Titin immunoglobulin domain protein antibody
    • TTID antibody
    • TTID protein antibody
    see all

Anti-Myotilin antibody 图像

  • Anti-Myotilin antibody (ab68915) at 1 µg/ml + Skeletal Muscle (Rat) Tissue Lysate at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution

    Performed under reducing conditions.

    Predicted band size : 56 kDa
    Observed band size : 56 kDa


    Exposure time : 2 minutes
  • IHC image of ab68915 staining in skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab68915, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

    For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

Anti-Myotilin antibody (ab68915)参考文献

This product has been referenced in:
  • Lin X  et al. Z-disc-associated, alternatively spliced, PDZ motif-containing protein (ZASP) mutations in the actin-binding domain cause disruption of skeletal muscle actin filaments in myofibrillar myopathy. J Biol Chem 289:13615-26 (2014). Read more (PubMed: 24668811) »

See 1 Publication for this product

Product Wall

Abcam has not validated the combination of species/application used in this Abreview.
Application Western blot
Sample Mouse Tissue lysate - whole (Heart)
Gel Running Conditions Reduced Denaturing (4-12%)
Loading amount 10 µg
Specification Heart
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
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Abcam user community

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提交于 Aug 22 2016

Abcam has not validated the combination of species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Mouse Tissue (Embryonic Heart Cryo-Section)
Permeabilization Yes - Tween-20
Specification Embryonic Heart Cryo-Section
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Fixative Paraformaldehyde
Username

Abcam user community

Verified customer

提交于 Jul 25 2016

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