Overview

  • Product name

  • Description

    Rabbit polyclonal to Myotilin
  • Host species

    Rabbit
  • Tested applications

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

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

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

    Read Abcam's proprietary immunogen policy (Peptide available as ab74353.)

  • Positive control

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

Properties

Applications

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.

Application Abreviews Notes
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).

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

  • 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.

References

This product has been referenced in:

  • Riedl I  et al. Association of the ACTN3 R577X polymorphism with glucose tolerance and gene expression of sarcomeric proteins in human skeletal muscle. Physiol Rep 3:N/A (2015). Read more (PubMed: 25780092) »
  • 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 all 2 Publications for this product

Customer reviews and Q&As

1-4 of 4 Abreviews or Q&A

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

Abcam user community

Verified customer

Submitted Aug 22 2016

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

Abcam user community

Verified customer

Submitted Jul 25 2016

Question
Answer

Thank you for your phone call.

DISCOUNT CODE: xxx
Expiration date: xxx

I am very pleased to hear you would like to accept our offer and test ab68915 in human and IHC-Fr. This code will give you: 1 free PRIMARY ANTIBODY before the expiration date. To redeem this offer, please submit an Abreview for human and IHC-Fr and include this code in the “Additional Comments” section so we know the Abreview is for this promotion. Please remember that submission of the Abreview is sufficient for the discount code to become active.
For more information on how to submit an Abreview, please visit the site: www.abcam.com/Abreviews.

Remember, we publish both positive and negative Abreviews on our datasheets so please submit the results of your tests. The code will be active once the Abreview has been submitted and can be redeemed in one of the following ways: 1) Call to place your order and mention the code to our customer service department; 2) Include the code in your fax order; 3) Place your order on the web and enter the promotional code.

Any feedback that you can provide will be greatly appreciated, whether positive or negative. If you have any further questions, please do not hesitate to contact us. We look forward to receiving your Abreview and wish you luck with your research.

The terms and conditions applicable to this offer can be found here: https://www.abcam.com/collaborationdiscount.

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Answer

Thank you for your enquiry which has been forwarded to the scientific support team. I am sorry we do not provide larger vial sizes. We can offer a discount if you purchase 5 vials or more on one order (of the same product, or different products). I can recommend to contact our customer service department with a completed  list of which antibodies and the quantity required for each product. They will be happy to then provide you with a quote. Please email: orders@abcam.com Thank you for your interest in our products. If you have any further questions, please do not hesitate to contact us.

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