The Expression And Potential Role Of Syncoilin Andβ-synemin In Duchenne Muscular Dystrophy

Objective The purposes are to analyze the expression of Syncoilin and beta-Synemin in skeletal muscle fiber cytoplasm of DMD patients, and get further insight into the pathogenesis; to investigate the relationship between the age of diagnosis of DMD patients, the degree of pathology, the degree of clinical significance with Syncoilin and beta-Synemin, and clarify the clinic value of two proteins.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 patients40cases present on deficency of dystrophin protein. The case-control group contain32cases with clinical and skeletal muscle pathological diagnosis. The control group30cases select patients from orthopedics who suffer acute trauma without neuromuscular disease. By immunohistochemical staining, the image-proplus6.0image analysis software detects groups of Syncoilin and beta-Synemin 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 Syncoilin and beta-Synemin.Results1. Clinical analysis:all of40cases 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 Syncoilin and beta-Synemin 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. Syncoilin and beta-Synemin protein missing in DMD patients muscluar fiber cytoplasm.2. With the degree of pathology and the degree of clinical significance severity, the deficiencies of Syncoilin and beta-Synemin gradually increased, suggesting that these two protein involved in the pathogenesis of DMD.