ABSTRACT
<p><b>OBJECTIVE</b>To investigate effect of CD4(+) CD25(+) Foxp3(+) Tregs in the treatment of autoimmune myositis (EAM) in mice and explore the possible mechanisms.</p><p><b>METHODS</b>Mouse models of EAM were divided randomly into model group and treatment group, and the latter received infusion of CD4(+) CD25(+) Foxp3(+) Tregs separated from normal mouse spleen by magnetic activated cell sorting. The changes of muscle pathology was observed, and the expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs was analyzed using flow cytometry; peripheral blood IL-10 and TGF-β levels were tested using double antibody sandwich ELISA.</p><p><b>RESULTS</b>Compare with the model group, the mice in the treatment group showed significantly reduced muscular inflammatory cell infiltration, increased blood levels of IL-10 and TGF-β (P<0.05), and increased expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs (P<0.05).</p><p><b>CONCLUSION</b>CD4(+) CD25(+) Foxp3(+) Tregs reinfusion produces therapeutic effect in mice with EAM by increasing peripheral blood IL-10 and TGF-β levels and PD-1 and CTLA-4 expressions in spleen CD4(+) CD25(+) Foxp3(+) Tregs.</p>
Subject(s)
Animals , Mice , Autoimmune Diseases , Allergy and Immunology , CTLA-4 Antigen , Metabolism , Cell Separation , Cell- and Tissue-Based Therapy , Disease Models, Animal , Flow Cytometry , Interleukin-10 , Blood , Myositis , Allergy and Immunology , Programmed Cell Death 1 Receptor , Metabolism , Spleen , Allergy and Immunology , T-Lymphocytes, Regulatory , Allergy and Immunology , Transforming Growth Factor beta1 , BloodABSTRACT
<p><b>OBJECTIVE</b>To compare the clinical, pathological, laboratory test and follow-up data between familial and sporadic patients with distal myopathy with rimmed vacuoles (DMRV) and discuss the characteristics of this disorder in Chinese population.</p><p><b>METHODS</b>The clinical and pathological features, laboratory data and follow-up results of 33 sporadic and 4 familial cases of pathologically confirmed DMRV were summarized and compared retrospectively.</p><p><b>RESULTS</b>The patients age, onset age, or disease duration showed no significant difference between sporadic and familial cases; the onset pattern and affected muscle groups were also similar, but the sporadic cases showed more frequent dysmorphic features than the familial cases. The patients showed mild to moderate elevation of the muscle enzymes by one to three folds, and the familial patients had more significant elevation than the sporadic ones. No correlation was found between the disease duration and the level of muscle enzymes. The pathological findings were similar between the cases, and Gomori staining showed rimmed vacuoles and inclusion bodies without inflammatory cell infiltration. Follow-up results of 29 cases showed no significant difference between the two groups. The disease was slowly progressive and severely affected the quality of life of the patients, but did not produce obvious effect on the life expectancy.</p><p><b>CONCLUSION</b>The clinical, pathological and laboratory data of Chinese DMRV patients are basically similar to those of Japanese cases. Sporadic cases tend to show more dysmorphic features than the familial ones, and occasional sporadic cases have early disease onset in early childhood.</p>
Subject(s)
Adult , Female , Humans , Male , Young Adult , Asian People , Distal Myopathies , Classification , Genetics , Pathology , Inclusion Bodies , Pathology , Pedigree , Retrospective Studies , Vacuoles , PathologyABSTRACT
0.05).Conclusions The expression of myostatin gene mRNA is increased in myotonic dystrophy.Up-regulated expression of myostatin in skeletal muscle might be associated with the mechanism of myotonic dystrophy.