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Up-regulation of MHC class I expression accompanies but is not required for spontaneous myopathy in dysferlin-deficient SJL/J mice.

Abstract
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.
AuthorsChristine A Kostek, Janice A Dominov, Jeffrey Boone Miller
JournalThe American journal of pathology (Am J Pathol) Vol. 160 Issue 3 Pg. 833-9 (Mar 2002) ISSN: 0002-9440 [Print] United States
PMID11891182 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • DYSF protein, human
  • Dysferlin
  • Histocompatibility Antigens Class I
  • Membrane Proteins
  • Muscle Proteins
Topics
  • Animals
  • Dysferlin
  • Gene Deletion
  • Histocompatibility Antigens Class I (biosynthesis, genetics)
  • Membrane Proteins
  • Mice
  • Mice, Inbred C57BL
  • Muscle Proteins (deficiency, genetics)
  • Muscular Dystrophies (etiology, genetics, metabolism)
  • Up-Regulation

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