Résumé
<p><b>OBJECTIVE</b>To establish a specific technique for diagnosing and classifying Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), facioscapulohumeral muscular dystrophy (FSHD) and neurologic dystrophy.</p><p><b>METHODS</b>Forty-seven cases were detected by immunofluorescence technique for analyzing dystrophin located in skeletal muscle cell membrane with the use of mouse monoclonal antibodies, goat and rabbit polyclonal antibodies.</p><p><b>RESULTS</b>The normal individuals showed ringed positive staining stripe around muscle fibers. Negative result of staining was seen in 16 DMD patients. Eleven BMD patients had discontinuous or a patchy positive staining pattern, and all of 10 FSHD and 10 neurological amyotrophic patients showed positive dystrophin staining.</p><p><b>CONCLUSION</b>Detecting dystrophin in the skeletal muscle cell membrane of muscular patients is an efficient technique for diagnosing and classifying various types of muscular dystrophy.</p>
Sujets)
Adolescent , Adulte , Enfant , Enfant d'âge préscolaire , Femelle , Humains , Mâle , Diagnostic différentiel , Dystrophine , Technique d'immunofluorescence , Méthodes , Muscles squelettiques , Chimie , Anatomopathologie , Dystrophies musculaires , Diagnostic , Métabolisme , Myopathie de Duchenne , Diagnostic , Métabolisme , Dystrophie musculaire facio-scapulo-humérale , Diagnostic , Métabolisme , Maladies neuromusculaires , Diagnostic , MétabolismeRésumé
AIM: To observe skeletal muscle damage of mdx mice after overload exercise, and protection to muscle damage induced by exercise due to myoblast transplantation (MTT). METHODS: Muscle samples of C 57 mice were minced and digested with trypsin, and myoblasts were cultured ex vivo , purified and detected by immunohistochemistry stains. The myoblasts were injected into muscle of left limb of mdx mice, whereas the right limb was injected with DMEM liquid as control. Mice were submitted to exercise for 3 days starting 1 month after MTT, and then Evans blue was injected intravenously through the tail vein. The muscle cryostat sections of mdx mice were made, and then detected the immunofluorescence of dystrophin. Under a fluorescence microscope, the number of fiber stained with Evans blue and dystrophin was counted, analyzed quantitatively with image software. RESULTS: Under a fluorescence microscope, only 10 37%?2 87% muscle fibers in the myoblast grafted muscles were stained with Evans blue. In contrast, 26 82%?14 85% muscle fibers in right control muscles were stained. Significant differences between these two groups were showed ( P