Up-Regulation of MHC Class I Expression Accompanies but Is Not Required for Spontaneous Myopathy in Dysferlin-Deficient SJL/J Mice
| Autor: | Janice A. Dominov, Jeffrey Boone Miller, Christine A. Kostek |
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| Jazyk: | angličtina |
| Rok vydání: | 2002 |
| Předmět: |
Ratón
Muscle Proteins chemical and pharmacologic phenomena Major histocompatibility complex Muscular Dystrophies Pathology and Forensic Medicine Dysferlin Pathogenesis Mice immune system diseases MHC class I medicine Animals Muscular dystrophy Myopathy biology Histocompatibility Antigens Class I Membrane Proteins hemic and immune systems medicine.disease Molecular biology nervous system diseases Up-Regulation Mice Inbred C57BL Immunology biology.protein medicine.symptom Gene Deletion Limb-girdle muscular dystrophy Regular Articles |
| Popis: | We found that up-regulation of major histocompatibility complex (MHC) class I expression accompanies, but is not required for, appearance of spontaneous myopathy in SJL/J mice. In some neuromuscular diseases, MHC class I expression is markedly up-regulated in muscles, though the consequences of this up-regulation for pathology are not clear. To study MHC class I in myopathy, we compared muscles of SJL/J mice to muscles of SJL/J mice that were also MHC class I-deficient due to targeted mutation in the beta-2-microglobulin gene (SJL/J B2m (-/-) mice). SJL/J mice show spontaneous myopathy and have a mutation in the dysferlin gene, a gene which is also mutated in human limb-girdle muscular dystrophy type 2B (LGMD2B). Muscles of eight-month-old SJL/J mice had higher levels of MHC class I expression than muscles of either C57BL/6J (wild-type) or SJL/J B2m (-/-) mice. In contrast, the percentage of abnormal muscle fibers was similar in SJL/J and SJL/J B2m (-/-) muscles. Invading Mac-1(+) cells were most abundant in SJL/J B2m (-/-) muscles, moderately abundant in SJL/J muscles, and rare in C57BL/6J muscles. Thus, MHC class I was markedly up-regulated in SJL/J muscles, but this high level of MHC class I was not necessary for the appearance of myopathy. |
| Databáze: | OpenAIRE |
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