Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin: An immunopathological study

Paolo Confalonieri, Laura Oliva, Francesca Andreetta, Rossella Lorenzoni, Patrizia Dassi, Elisabetta Mariani, Lucia Morandi, Marina Mora, Ferdinando Cornelio, Renato Mantegazza

Research output: Contribution to journalArticlepeer-review

Abstract

Muscle inflammation is characteristic of inflammatory myopathies but also occurs in muscular dystrophy with lack of the sarcolemmal protein dysferlin. We quantified inflammatory cells and major histocompatibility complex (MHC) expression in muscle from 10 patients with dysferlinopathy. Infiltrating cells were always present although numbers varied considerably; macrophages were more common than T cells, T cytotoxicity was absent, and MHC class I was overexpressed on muscle fibers. These findings differ from polymyositis (PM) but are closely similar to those in SJL/J mice (which lack dysferlin) and emphasize the relationship between absence of dysferlin and immune system abnormalities in muscle.

Original languageEnglish
Pages (from-to)130-136
Number of pages7
JournalJournal of Neuroimmunology
Volume142
Issue number1-2
DOIs
Publication statusPublished - Sep 2003

Keywords

  • Dysferlin
  • Immunohistochemical analysis
  • Muscle inflammation
  • Muscular dystrophy

ASJC Scopus subject areas

  • Immunology
  • Clinical Neurology
  • Immunology and Allergy
  • Neurology

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