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(American Journal of Pathology. 2001;158:355-359.)
© 2001 American Society for Investigative Pathology

Short Communication

X-Linked Vacuolated Myopathy

TNF- and IFN- Expression in Muscle Fibers with MHC Class I on Sarcolemma

Karl Rouger^*, Jean-Pierre Louboutin^*, Marcello Villanova, Yan Cherel and Michel Fardeau^§

From INSERM U533 Laboratoire de Physiopathologie et Pharmacologie Cellulaires et Moleculaires-Hotel Dieu,^*
Nantes, France; the Institute of Orthopaedics Rizzoli,
Bologna, Italy; the Service d’Anatomo-Pathologie-Ecole Nationale Veterinaire,
Nantes, France; and the Institut de Myologie,^§
Paris, France

The presence and the distribution of tumor necrosis factor-, interferon-, and p65 subunit of nuclear factor-B, 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- and interferon-, 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- and interferon- and partly mediated by nuclear factor-B.

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