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  • Title: A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice. Immunological evidence.
    Author: Pons F, Augier N, Léger JO, Robert A, Tomé FM, Fardeau M, Voit T, Nicholson LV, Mornet D, Léger JJ.
    Journal: FEBS Lett; 1991 Apr 22; 282(1):161-5. PubMed ID: 1709117.
    Abstract:
    Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of Mr 400,000 which is present in extracts of mdx muscle from regions which contain neuromuscular junctions (NMJ) and is absent from those which do not. This NMJ-associated homologue of dystrophin has at least 2 epitopes which are different to usual Xp21 form of dystrophin expressed along the sarcolemma of muscle fibres in normal muscles. This protein is also expressed at the NMJ of a DMD patient who lacks the first 52 exons of the Xp21 dystrophin gene and it must therefore be translated from a different gene transcript.
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