The Expression And Clinical Research Of α-Sytrophin And α-Dystrobrevin In Duchenne Muscular Dystrophy

Objective The purpose is to analyze the expression of alpha-Syntrophin and alpha-Dystrobrevin in skeletal muscle sarcolemma of DMD patients, further insight into the pathogenesis; Investigate the relationship between the age of diagnosis of DMD patients, the degree of pathology, the degree of clinical significance and alpha-Syntrophin and alpha-Dystrobrevin to clarify the value of clinic.Methods Select patients clinically suspected DMD、Limb-girdle dystrophy-2B(LGMD2B)、patients from orthopedics who suffer acute trauma without neuromuscular disease, treated by open skeletal muscle biopsy. DMD patients52cases present on deficency of dystrophin protein. The case-control group contain48cases with clinical and skeletal muscle pathological diagnosis. The control group46cases select patients from orthopedics who suffer acute trauma without neuromuscular disease. By immunohistochemical staining, the image-proplus6.0image analysis software detects groups of alpha-Syntrophin and alpha-Dystrobrevin expression, use integral optical density(IOD) said, comparations between the two groups, analyze the relationship between diagnosed age,the degree of pathology,the degree of clinical significance with alpha-Syntrophin、alpha-Dystrobrevin.Results1. Clinical analysis:all of52cases are male, average diagnosis age of is7.1±3.2years olds; The main clincal manifestations are myasthenia, for example, walking unsteadily, easy failing, going up staircase difficultly.2. Histochemical staining findings:all DMD pateints showed typical muscular dystrophy.3. Immunohistochemical staining results:IOD of alpha-Syntrophin and alpha-Dystrobrevin among the three groups(the DMD group,case-control group,normal control group) are different significance(P<0.05). The DMD group and case-control group are significant differences statistically(P<0.05); The DMD group and normal control group are significant differences statistically(P<0.05); the case-control group and normal control group aren’t significant differences statistically(P>0.05). There are not significant differences statistically with diagnosed age(P>0.05), and are significantly different in the degree of pathology and the degree of clinical significance (P<0.05).Conclusions1. Alpha-Syntrophin and alpha-Dystrobrevin protein missing in DMD patients muscluar fiber membrane.2. With the degree of pathology and the degree of clinical significance severity, the deficiencies of alpha-Syntrophin and alpha-Dystrobrevin gradually increased, suggesting that these two protein involved in the pathogenesis of DMD.