Research Of Characters Of Neuromyopathy Patients' Skeletal Muscle Fibers Regeneration And Expression And Significance Of Ezrin And PI3K In Regenerating Muscle Fibers

Neuromuscular diseases are diseases of the nervous system in a category with very high morbidity. Pathology study found that skeletal muscle regeneration could prevent the loss of muscle volume and ensure the maintenance of normal muscle morphology and function after injury. Researching muscle fiber regeneration characteristics and muscle fiber regeneration relevant factors, it was of great significance for study and treatment of neuromuscular myopathy.Based on this study of muscle fiber regeneration of the current research base, a long-term clinical work to collect a large number of patients with myopathy of skeletal muscle biopsy specimens, including: DMD and BMD, DM, PM, ALS, SMA, and so on. Application histochemistry stain, Immunohistochemistry stain, histomorphometry methods, statistical approach to study various state of skeletal muscle myopathy and pathological features of muscle fiber regeneration. Through electron microscopy observated the ultrastructure of muscle satellite cells and regeneration of muscle fiber, and clearly defined muscle fiber regeneration characteristics of motor neuron disease. This experiment was the first time Ezrin, PI3K proteins applied to patients with myopathy to research skeletal muscle fiber regeneration by histochemistry stain, Immunohistochemistry stain. Observed protein expression of homology regenerating muscle fiber to approach these two proteins associated with the muscle fiber regeneration mechanism. This study for the awareness and promoting myopathy state muscle fibers regenerate, provided a theoretical basis for myopathy therapy.1,Pathological and chemical characteristics of skeletal muscle regeneration of myopathy patientsCollected skeletal muscle specimens and clinical data of muscular dystrophy, motor neuron disease, inflammatory myopathy and other neurological patients, using histochemical stain, immunohistochemistry stain, histomorphometry and statistical method to research muscle fiber regeneration of different myopathy situation, and marker of muscle fiber regeneration NCAM marked regeneration of muscle fiber, summed up the characteristics of regenerating fiber.Results: HE staining of DMD, DM, PM, BMD, SMA were found skeletal muscle regeneration muscle fibers were smaller diameter,bigger and round nuclear, basophilic cytoplasm, NCAM staining saw deep into muscle fiber regeneration. DMD inflammatory myopathy and muscle fiber regeneration obvious than other active myopathy. Morphometric study found that DMD patients younger, more active muscle fiber regeneration.Conclusions: Muscle fiber regeneration of DMD, DM and PM in patients was active, BMD muscle fiber regeneration relatively small. Motor neuron disease ALS no obvious regenerating muscle fibers; SMA small amount of muscle fiber regeneration. Muscle fibers regeneration close to the inflammatory response. Inflammatory response could promote muscle regeneration. Muscular dystrophy and inflammatory myopathy regeneration might be the same mechanism, involved in the inflammatory response; motor neuron disease was different from the regeneration mechanism. DMD patients with skeletal muscle fiber regeneration and age correlation, the more active younger renewable, renewable gradually diminish with increasing age.2,Ultrastructure study of skeletal muscle regeneration of neural myopathy patientsIn histochemical stain and immunohistochemical stain, muscle fiber regeneration of muscular dystrophy and inflammatory myopathy is active, patients with neurogenic changes in skeletal muscle regeneration was not active, ALS was not even found renewable fiber, SMA only a small number of small diameter fiber regeneration. To further explore muscle fiber regeneration, collecting skeletal muscle specimens of patients with myopathy to study ultrastructure.Results: DMD, limb-girdle muscular dystrophy, CMT and neurogenic changes in the skeletal muscle specimens under the electronic microscope see the activation and splitting the muscle satellite cells and regeneration of muscle fiber.Conclusions: Patients with neurogenic changes in the muscle had active and splitting muscle satellite cell, early regenerating muscle fibers, regenerating fiber could not development mature. Muscular dystrophy patients were been seen static and splitting satellite cells, early regenerating muscle fiber and inflammatory response is active.3,Expression and significance of Ezrin in skeletal muscle fiber regeneration of patients with myopathyUnder optical microscopy, selected active regeneration skeletal muscle specimens,series frozen section and through the method of histochemical, immunohistochemical staining observed homology regeneration muscle fiber and analysis Ezrin expression in the regenerating muscle fibers .Results: Anti-Ezrin antibody staining in DMD, PM muscle, compared homology muscle cells in HE and NCAM staining,regenerating muscle fibers deeply stained, small diameter and early renewable fiber coloring deep, mature muscle fibers do not painted, with the muscle fibers mature , dyeing gradually weakened to disappear. Anti-Ezrin,anti-NCAM antibody staining, sections of two homologous staining, anti-NCAM staining of regenerating muscle fibers and myoblasts were positive, anti-Ezrin antibody staining showed deep.Conclusions: Ezrin protein had close relation with skeletal muscle fiber regeneration of myopathy patients.In the early myoblast cell differentiation and migration process, speculating Ezrin mediated that CD44 connectted with cells, and promoted myoblast migration and differentiation; myoblast fusion period, the calpain might hydrolysis cytoskeleton structure such as Ezrin, vimentin and made muscle cells instability, more conducive to integration, forming muscle fibers; in the muscle fiber regeneration process , Ezrin might activate PI3K to promot the muscle fiber protein synthesis, thereby promoting the muscle fibers mature; Ezrin might synergy inflammatory response to promote muscle fiber regeneration.4,Expression and significance of PI3K in skeletal muscle fiber regeneration of patients with myopathyUnder optical microscopy, selected active regeneration skeletal muscle specimens, series frozen section and through the method of histochemical, immunohistochemical staining observed homology regeneration muscle fiber and analysis PI3K expression in the regenerating muscle fiber .Results:. PI3K in early renewable fiber membrane deep coloring, renewable fiber membrane of the larger the diameter staining slightly, mature muscle fibers and cytoplasm no PI3K expression. PI3K in arteriovenous walls deeply stainedConclusions: PI3K in regeneration skeletal muscle fibers of patients with myopathy early played an important role. In skeletal muscle fiber regeneration process of patients with myopathy,PI3K might transit signal transduction role to promot childish muscle fiber regeneration, protein synthesis, helped muscle fibers gradually mature. PI3K could be used as a new marker of muscle fiber regeneration.