[Presence of dystrophine-like protein at the neuromuscular junction in Duchenne muscular dystrophy and in "mdx" mutant mice].
We have studied by indirect immunofluorescence, using three different polyclonal antidystrophin antibodies raised against fusion proteins, the neuromuscular junctions (NMJs) in muscle biopsies from Duchenne muscular dystrophy (DMD) patients, from human controls and mutant "mdx" mice and normal mice. In controls the periphery of all muscle fibres was strongly labelled by the three dystrophin antibodies and there was a high concentration of labelling at the NMJs (where it was co-localized with acetylcholine receptor labelled by the alpha-bungarotoxin). In DMD and in "mdx" mice the NMJs were equally labelled whereas there was an absence of reaction at the periphery of all (DMD) or most ("mdx" mice) muscle fibers. These findings suggest that a dystrophin-like protein, which was identified by the antibodies we have used, is present at the NMJs in the Duchenne's myopathy and "mdx" mice.
Fardeau
M
M
I.N.S.E.R.M. U. n 153, Paris.
Tomé
F M
FM
Collin
H
H
Augier
N
N
Pons
F
F
Léger
J
J
Léger
J
J
D000818:Animals; D000906:Antibodies; D002648:Child; D002675:Child, Preschool; D016189:Dystrophin / Q000032:analysis* / Q000276:immunology; D005455:Fluorescent Antibody Technique; D006801:Humans; D051379:Mice; D008817:Mice, Mutant Strains; D009124:Muscle Proteins / Q000032:analysis* / Q000276:immunology; D009136:Muscular Dystrophies / Q000378:metabolism*; D009469:Neuromuscular Junction / Q000737:chemistry*
D000906:Antibodies; D016189:Dystrophin; D009124:Muscle Proteins
Présence d'une protéine de type dystrophine au niveau de la jonction neuromusculaire dans la dystrophie musculaire de Duchenne et la souris mutante "mdx".