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 language | English |
|---|---|
| Pages (from-to) | 130-136 |
| Number of pages | 7 |
| Journal | Journal of Neuroimmunology |
| Volume | 142 |
| Issue number | 1-2 |
| DOIs | |
| Publication status | Published - Sep 2003 |
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Keywords
- Dysferlin
- Immunohistochemical analysis
- Muscle inflammation
- Muscular dystrophy
ASJC Scopus subject areas
- Immunology
- Clinical Neurology
- Immunology and Allergy
- Neurology
Cite this
Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin : An immunopathological study. / Confalonieri, Paolo; Oliva, Laura; Andreetta, Francesca; Lorenzoni, Rossella; Dassi, Patrizia; Mariani, Elisabetta; Morandi, Lucia; Mora, Marina; Cornelio, Ferdinando; Mantegazza, Renato.
In: Journal of Neuroimmunology, Vol. 142, No. 1-2, 09.2003, p. 130-136.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin
T2 - An immunopathological study
AU - Confalonieri, Paolo
AU - Oliva, Laura
AU - Andreetta, Francesca
AU - Lorenzoni, Rossella
AU - Dassi, Patrizia
AU - Mariani, Elisabetta
AU - Morandi, Lucia
AU - Mora, Marina
AU - Cornelio, Ferdinando
AU - Mantegazza, Renato
PY - 2003/9
Y1 - 2003/9
N2 - 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.
AB - 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.
KW - Dysferlin
KW - Immunohistochemical analysis
KW - Muscle inflammation
KW - Muscular dystrophy
UR - http://www.scopus.com/inward/record.url?scp=12144287801&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=12144287801&partnerID=8YFLogxK
U2 - 10.1016/S0165-5728(03)00255-8
DO - 10.1016/S0165-5728(03)00255-8
M3 - Article
C2 - 14512171
AN - SCOPUS:12144287801
VL - 142
SP - 130
EP - 136
JO - Journal of Neuroimmunology
JF - Journal of Neuroimmunology
SN - 0165-5728
IS - 1-2
ER -