Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study aimed to investigate immunostaining patterns for major histocompatibility complex class I (MHC-I) in different types of myopathies and to assess the diagnostic value of CD8/MHC-I complex for definite polymyositis. The study included 20 cases of definite polymyositis, 20 cases of dermatomyositis and 10 cases of dystrophies with muscle inflammation (inflammatory muscular dystrophy). Immunohistochemical staining with MHC-I antibody demonstrated the presence of MHC-I along the sarcolemma of scattered or small groups of non-necrotic fibres in both polymyositis and inflammatory muscular dystrophy, whereas intense sarcolemmal immunostaining for MHC-I was diffusely present in almost all fibres in dermatomyositis. Double immunofluorescence labelling for CD8 and MHC-I detected the CD8/MHC-I complex in 20% of polymyositis cases with mononuclear cell infiltrates. No CD8/MHC-I complex was found in the dermatomyositis or inflammatory muscular dystrophy cases. The results suggest that MHC-I detection alone cannot be used as a reliable diagnostic test to differentiate polymyositis from dystrophies with secondary muscle inflammation. The CD8/MHC-I complex, although showing high specificity, is neither a sensitive nor an easy-to-handle diagnostic test for polymyositis.
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PMID:CD8/MHC-I complex is specific but not sensitive for the diagnosis of polymyositis. 2081 42

Human leukocyte antigen (HLA)-E is a non-classical major histocompatibility complex class I (Ib) molecule, which plays an important role in immunosuppression. In this study, we investigated the immunomodulating effect of HLA-E in a xenogeneic system, using human placental artery-derived endothelial (hPAE) cells expressing HLA-E in a mouse model. In vitro cell lysis analysis by primed lymphocytes in combination with siRNA transfection showed that HLA-E is necessary for inhibition of the immune response. Similarly, in vivo cell implantation analysis with siRNA-mediated down-regulation of HLA-E demonstrates that HLA-E is involved in immunosuppression. As hPAE cells efficiently transdifferentiate into myoblasts/myocytes in vitro, we transplanted the cells into mdx mice, a model of Duchenne muscular dystrophy. hPAE cells conferred dystrophin to myocytes of the 'immunocompetent' mdx mice with extremely high efficiency. These findings suggest that HLA-E-expressing cells with a myogenic potential represent a promising source for cell-based therapy of patients with muscular dystrophy.
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PMID:Dystrophin conferral using human endothelium expressing HLA-E in the non-immunosuppressive murine model of Duchenne muscular dystrophy. 2094 60