title: X-linked vacuolated myopathy : TNF-alpha and IFN-gamma expression in muscle fibers with MHC class I on sarcolemma.
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.
authors.affiliation: INSERM U533 Laboratoire de Physiopathologie et Pharmacologie Cellulaires et Moleculaires-Hotel Dieu, Nantes, France.
authors.forename: K
authors.initials: K
authors.lastname: Rouger
authors.forename: J P
authors.initials: JP
authors.lastname: Louboutin
authors.forename: M
authors.initials: M
authors.lastname: Villanova
authors.forename: Y
authors.initials: Y
authors.lastname: Cherel
authors.forename: M
authors.initials: M
authors.lastname: Fardeau
mesh_terms: D005786:Gene Expression Regulation; D008040:Genetic Linkage; D015395:Histocompatibility Antigens Class I / Q000032:analysis / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D007371:Interferon-gamma / Q000032:analysis / Q000235:genetics; D018485:Muscle Fibers, Skeletal / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D009135:Muscular Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016328:NF-kappa B / Q000032:analysis / Q000235:genetics; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis / Q000235:genetics; D014960:X Chromosome / Q000235:genetics*
chemical_list: D015395:Histocompatibility Antigens Class I; D016328:NF-kappa B; D012333:RNA, Messenger; D014409:Tumor Necrosis Factor-alpha; D007371:Interferon-gamma