The Expression Of Sarcoglycans In Patients With Progessive Muscular Dystrophy And Its Clinical Pathological Analysis

Objective: To observe the character and percentage of sarcoglycans expression on the sarcolemma of muscle fibers between DMD and BMD patients.To explain the relationship between dystrophin and sarcoglycans and to illuminate the mechanism of muscular dystrophy at the level of protein.To identify the relationship expression of SGs in muscle fibers with their clinical profiles and muscle pathological changes.Methods: Biceps brachii is treated by muscular biopsy.All patients are divided into two groups, the case group and the control group. The case group includes DMD and BMD patients diagnosed by the immunohistochemistical stain of anti-dystrophin-N,-C,-R monoclone antibody. DMD patients 21 cases present on the complete deficency of dystrophin protein on the sarcolemma of muscle fibers. BMD patients 12 cases present on the partial deficency of dystrophin protein on the sarcolemma of muscle fibers. The case group are observed the character of histochemical and deaminase stain under the optic microscope.The control group include the spinal muscular atrophy 10 cases, PM 7 cases, DM 7 cases , IBM 6 cases and normal muscle 3 cases, which are diagnosed by the clinical and pathologic diagnostic criteria .All the patients are treated with immunohistochemical stain of anti-α-,β-,γ-,δ-sarcoglycan monoclone antibody. To observe the deficency and percentage ofα-,β-,γ-,δ-sarcoglycan on the sarcolemma of muscle fibers under the optic microscope. The results of qualitative analysis are dealed with the statistic analysis ofχ2 test .Result: The clinical changes of 33 cases 1-3 are 16 cases, 4-5 are 17 cases, the deficency of sarcoglycans on the sarcolemma of muscle fibers is 60.93% and 80.88%, the clinical changes is more serious , the deficency of sarcoglycans is severer(P﹤0.05).The muscle pathology changes of 33 cases 10%-40% are 14 cases,40%-70% are 19 cases , the deficency of sarcoglycans on the sarcolemma of muscle fibers is 55.36% and 82.90%, the muscle pathology changes is more more serious , the deficency of sarcoglycans is severer(P﹤0.05). The deficency of sarcoglycans on the sarcolemma of muscle fibers of DMD patients is more serious comparing to that of BMD patients(P﹤0.05).The deficency ofα-sarcoglycan protein on the sarcolemma of muscle fibers is more serious comparing toβ-,γ-,δ-sarcoglycan protein of DMD patients and BMD patients(P﹤0.05),but there were no significant differences ofβ-,γ-,δ-sarcoglycan (P﹥0.05).Conclusion: The clinical and muscle pathology changes is more serious , the deficency of sarcoglycans is severer ,maybe this can explain the deficiency of sarcoglycans increase the degenerating and regenerating of fibers. DMD and BMD patients partly have the deficency of sarcoglycans,but that of DMD patients is more serious to explain the deficency of dystrophin can defect the expression of sarcoglycans.When the dystrophin have the complete deficency, the expression of sarcoglycans are less . The deficency ofα-sarcoglycan protein on the sarcolemma of muscle fibers is more serious comparing toβ-,γ-,δ-sarcoglycan , maybe this is relation to the complex .