Overview

  • Product name

  • Description

    Rabbit polyclonal to Myotilin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to Human Myotilin aa 64-152.
    Sequence:

    MSSSAFPASPQQHAGSNPGQRVTTTYNQSPASFLSSILPSQPDYNSSKIP SAMDSNYQQSSAGQPINAKPSQTANAKPIPRTPDHEIQG


    Database link: Q9UBF9

  • Positive control

    • IHC-P: Human skeletal muscle tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab224540 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 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

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 skeletal muscle tissue stained for Myotilin with ab224540 at 1/200 dilution in immunohistochemical analysis.

References

ab224540 has not yet been referenced specifically in any publications.

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