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.
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.
authors.affiliation: Pathologie Générale, Faculté de Médecine, Montpellier, France.
authors.forename: F
authors.initials: F
authors.lastname: Pons
authors.forename: N
authors.initials: N
authors.lastname: Augier
authors.forename: J O
authors.initials: JO
authors.lastname: Léger
authors.forename: A
authors.initials: A
authors.lastname: Robert
authors.forename: F M
authors.initials: FM
authors.lastname: Tomé
authors.forename: M
authors.initials: M
authors.lastname: Fardeau
authors.forename: T
authors.initials: T
authors.lastname: Voit
authors.forename: L V
authors.initials: LV
authors.lastname: Nicholson
authors.forename: D
authors.initials: D
authors.lastname: Mornet
authors.forename: J J
authors.initials: JJ
authors.lastname: Léger
mesh_terms: D000818:Animals; D000906:Antibodies; D000911:Antibodies, Monoclonal / Q000276:immunology; D015153:Blotting, Western; D002648:Child; D016189:Dystrophin / Q000096:biosynthesis* / Q000235:genetics; D000939:Epitopes; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D051379:Mice; D009136:Muscular Dystrophies / Q000378:metabolism*; D009469:Neuromuscular Junction / Q000378:metabolism*
chemical_list: D000906:Antibodies; D000911:Antibodies, Monoclonal; D016189:Dystrophin; D000939:Epitopes