Overview

  • Product name
  • Description
    Goat polyclonal to Myotilin
  • Host species
    Goat
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide:

    DSQQHNSEHARLQ

    by a Cysteine residue linker, corresponding to internal sequence amino acids 209-221 of Human Myotilin (NP_006781.1; NP_001129412.1).

  • Positive control
    • Human skeletal muscle lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab156953 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
WB Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 60 kDa (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

  • Anti-Myotilin antibody (ab156953) at 0.1 µg/ml + Human skeletal muscle lysate at 35 µg

    Developed using the ECL technique.

    Predicted band size: 55 kDa
    Observed band size: 60 kDa (why is the actual band size different from the predicted?)

References

ab156953 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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