[Specificity of the dystrophin immunohistochemical technique in muscular dystrophy].
We have used a monoclonal antibody against the portion of the dystrophin molecule known as a mid rod using an immunoperoxidase technique in 41 muscle biopsies of patients with different muscle diseases. Twelve of the patients suffered a Xp21 dystrophy, nine of them had the Duchenne type and three the Becker. All the patients affected by a non Xp21 dystrophy had a normal dystrophin labelling pattern in their muscle fibres. The labelling patterns in the Duchenne biopsies were of three types. In seven of them we found no dystrophin at all. One showed only small nuclei of fibres with a normal amount of dystrophin in them. The last patient showed a weak labelling pattern in the majority of his fibres. In the two Becker dystrophy-type biopsies the expression of dystrophin varied. In one of them we could see a clear labelling pattern in all fibres, while the other showed a disruptive pattern with some portions of the sarcolemic membrane without dystrophin. We concluded the report enhancing the enormous support that this technique means particularly in the biopsies of patients suffering from an Xp21 alterations.
Unidad de Patología Neuromuscular, Hospital Sant Joan de Déu, Barcelona.
J
J
Colomer Oferil
J
J
Vila Torres
C
C
Huezo Montoya
D000911:Antibodies, Monoclonal / Q000276:immunology; D001706:Biopsy; D015153:Blotting, Western; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002891:Chromosomes, Human, Pair 21; D003937:Diagnosis, Differential; D016189:Dystrophin / Q000032:analysis / Q000276:immunology*; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D009132:Muscles / Q000737:chemistry / Q000166:cytology / Q000276:immunology; D009136:Muscular Dystrophies / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis
D000911:Antibodies, Monoclonal; D016189:Dystrophin
Especificidad de la técnica inmunohistoquímica de la distrofina en las distrofias musculares de Duchenne y de Becker.