Abstract
The presence and the distribution of tumor necrosis factor-alpha, interferon-gamma, and p65 subunit of nuclear factor-kappaB, molecules known to induce synergistically and to mediate major histocompatibility complex (MHC) class I expression, were determined in muscle sections from control and X-linked vacuolated myopathy patients. MHC class I colocalized with tumor necrosis factor-alpha and interferon-gamma, as well as with p65, in most of the membrane attack complex- and/or calcium-positive muscle fibers in X-linked vacuolated myopathy. These results suggest that the expression of MHC class I in X-linked vacuolated myopathy could be induced by tumor necrosis factor-alpha and interferon-gamma and partly mediated by nuclear factor-kappaB.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Gene Expression Regulation
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Genetic Linkage
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Histocompatibility Antigens Class I / analysis
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Histocompatibility Antigens Class I / genetics
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Humans
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Immunohistochemistry
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Interferon-gamma / analysis
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Interferon-gamma / genetics
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Muscle Fibers, Skeletal / chemistry
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Muscle Fibers, Skeletal / metabolism
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Muscle Fibers, Skeletal / pathology
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Muscular Diseases / genetics
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Muscular Diseases / metabolism
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Muscular Diseases / pathology*
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NF-kappa B / analysis
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NF-kappa B / genetics
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RNA, Messenger / genetics
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RNA, Messenger / metabolism
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Reverse Transcriptase Polymerase Chain Reaction
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Tumor Necrosis Factor-alpha / analysis
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Tumor Necrosis Factor-alpha / genetics
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X Chromosome / genetics*
Substances
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Histocompatibility Antigens Class I
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NF-kappa B
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RNA, Messenger
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Tumor Necrosis Factor-alpha
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Interferon-gamma