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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(s) -
Pons F.,
Augier N.,
Léger J.O.C.,
Robert A.,
Tome F.M.S.,
Fardeau M.,
Voit T.,
Nicholson L.V.B.,
Mornet D.,
Léger J.J.
Publication year - 1991
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(91)80468-i
Subject(s) - dystrophin , duchenne muscular dystrophy , sarcolemma , epitope , utrophin , neuromuscular junction , biology , polyclonal antibodies , exon , muscular dystrophy , microbiology and biotechnology , neuromuscular disease , gene , myocyte , antibody , genetics , neuroscience , medicine , disease
Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of M r 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 the 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.