Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin: an immunopathological study.
J Neuroimmunol
; 142(1-2): 130-6, 2003 Sep.
Article
em En
| MEDLINE
| ID: mdl-14512171
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Antígenos de Histocompatibilidade Classe I
/
Proteínas
/
Regulação para Cima
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Músculo Esquelético
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Proteínas Musculares
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Distrofias Musculares
Limite:
Adolescent
/
Adult
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Humans
/
Middle aged
Idioma:
En
Revista:
J Neuroimmunol
Ano de publicação:
2003
Tipo de documento:
Article