Overview

  • Product name

  • Description

    Rabbit polyclonal to Myotilin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Pig
  • Immunogen

    Recombinant fragment within Human Myotilin (internal sequence). The exact sequence is proprietary.
    Database link: Q9UBF9

  • Positive control

    • WB: A549 whole cell lysate. IHC: Human hepatoma tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab228763 in the following tested applications.

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

Application Abreviews Notes
IHC-P 1/100 - 1/1000.
WB 1/500 - 1/3000. Predicted molecular weight: 55 kDa.

Target

  • Function

    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.
  • Tissue specificity

    Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the myotilin/palladin family.
    Contains 2 Ig-like C2-type (immunoglobulin-like) domains.
  • Cellular localization

    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
  • Database links

  • Alternative names

    • 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

Images

  • Paraffin-embedded human hepatoma tissue stained for Myotilin using ab228763 at 1/500 dilution in immunohistochemical analysis.

  • Anti-Myotilin antibody (ab228763) at 1/3000 dilution + A549 (human lung carcinoma cell line) whole cell lysate at 30 µg

    Developed using the ECL technique.

    Performed under reducing conditions.

    Predicted band size: 55 kDa



    7.5% SDS-PAGE

References

ab228763 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab228763.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up