SJL/J mice have been studied as the model animals for autoimmunological diseases. Recently it was clarified that SJL/J mice have a defect of dysferlin. Human limb girdle muscular dystrophy 2B and Miyoshi myopathy also have a defect of dysferlin. In this study we present the histological and immunohistological changes in the natural course. Histological study revealed that SJL/J mice had inflammatory, degenerative changes, and neurogenic changes in later ages. As for interstitial inflammatory cells, the macrophages were dominant in any age, and in the T cell subset, the CD4+ T cells were more abundant than the CD8+ T cells, and few B cells were seen. The laboratory data showed a high level of creatine kinase in all ages. It is suspected that the inflammatory changes were induced by the primary immunological abnormality or by the defect of dysferlin in SJL/J mice.

Keywords:
SJL/J mice, Dysferlin, Inflammatory changes, Macrophage, Limb girdle muscular dystrophy 2B, Miyoshi myopathy
1.
Bernard CC, Carnegie PR: Experimental autoimmune encephalomyelitis in mice: immunologic response to mouse spinal cord and myelin basic proteins. J Immunol 1975;114:1537–1540.
2.
Dieperink ME, O’Neill A, Maselli R, Stefansson K: Experimental allergic neuritis in the SJL/J mice: dysfunction of peripheral nerve without clinical signs. J Neuroimmunol 1991;35:247–259.
3.
Rosenberg NL, Ringel SP, Kotzin BL: Experimental autoimmune myositis in SJL/J mice. Clin Exp Immunol 1987;68:117–129.
4.
Lerman SP, Chapman JM, Carswell EA, Thorbecke GJ: Properties of reticulum-cell sarcomas in SJL/J mice. I. Proliferative response to tumor cells of T-derived lymphoid cells from normal mice. Int J Cancer 1974;14:808–816.
5.
Liu J, Aoki M, Illa I, Wu C, Fardeau M, Angelini C, Serrano C, Urtizberea JA, Hentati F, Hamida MB, Bohlega S, Culper EJ, Amato AA, Bossie K, Oeltjen J, Bejaoui K, McKenna-Yasek D, Hosler BA, Schurr E, Arahata K, de Jong PJ, Brown RH Jr: Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy. Nat Genet 1998;20:31–36.
6.
Matsuda C, Aoki M, Hayashi YK, Ho MF, Arahata K, Brown RH Jr: Dysferlin is a surface membrane-associated protein that is absent in Miyoshi myopathy. Neurology 1999;53:1119–1122.
7.
Bashir R, Britton S, Strachan T, Keers S, Vafiadaki E, Lako M, Richard I, Marchand S, Bourg N, Argov Z, Sadeh M, Mahjneh I, Marconi G, Passos-Bueno M, Moreira Ede S, Zatz M, Beckmann JS, Bushby K: A gene related to Caenorhabditis elegans spermatogenesis factor fer-1 is mutated in limb-girdle muscular dystrophy type 2B. Nat Genet 1998;20:37–42.
8.
Bittner RE, Anderson LV, Burkhardt E, Bashir R, Vafiadaki E, Ivanova S, Raffelsberger T, Maerk I, Hoger H, Jung M, Karbasiyan M, Storch M, Lassmann H, Moss JA, Davison K, Harrison R, Bushby KM, Reis A: Dysferlin deletion in SJL/J mice (SJL-Dysf) defines a natural model for limb girdle muscular dystrophy 2B. Nat Genet 1999;23:141–142.
9.
Vafiadaki E, Reis A, Keers S, Harrison R, Anderson LV, Raffelsberger T, Ivanova S, Hoger H, Bittner RE, Bushby K, Bashir R: Cloning of the mouse dysferlin gene and genomic characterization of the SJL-Dysf mutation. Neuroreport 2001;12:625–629.
10.
Weller AH, Magliato SA, Bell KP, Rosenberg NL: Spontaneous myopathy in the SJL/J mouse: pathology and strength loss. Muscle Nerve 1997;20:72–82.
11.
Kostek CA, Dominov JA, Miller JB: Up-regulation of MHC class I expression accompanies but is not required for spontaneous myopathy in dysferlin-deficient SJL/J mice. Am J Pathol 2002;160:833–839.
12.
Matsubara S, Shima T, Takamari M: Experimental allergic myositis in SJL/J mice immunized with rabbit myosin B fraction. Acta Neuropathol (Berl) 1993;85:138–144.
13.
Nemoto H, Nemoto K, Sugimoto H, Kinoshita M: FK506 suppressed the inflammatory changes of EAM in SJL/J mice. J Neurol Sci 2001;193:7–11.
14.
Hohfeld R, Müller W, Toyka KV: Necrotizing myopathy in SJL/J mice. Muscle Nerve 1988;11:184–185.
15.
Beller DI, Springer TA, Schreiber RD: Anti-Mac-1 selectively inhibits the mouse and human type three complement receptor. J Exp Med 1982;156:1000–1009.
16.
Ho MK, Springer TA: Tissue distribution, structural characterization, and biosynthesis of Mac-3, a macrophage surface glycoprotein exhibiting molecular weight heterogeneity. J Biol Chem 1983;258:636–642.
17.
Lennon NJ, Kho A, Bacskai BJ, Perlmutter SL, Hyman BT, Brown RH: Dysferlin interacts with annexins A1 and A2 and mediates sarcolemmal wound-healing. J Biol Chem 2003;278:50466–50473.
18.
Rosenberg NL, Kotzin BL: Aberrant expression of class II MHC antigens by skeletal muscle endothelial cells in experimental autoimmine myositis. J Immunol 1989;142:4289–4294.
19.
Gallardo E, Rojas-Garcia R, de Luna N, Pou A, Brown RH Jr, Illa I: Inflammation in dysferlin myopathy: immunohistochemical characterization of 13 patients. Neurology 2001;57:2136–2138.
20.
Fanin M, Angelini C: Muscle pathology in dysferlin deficiency. Neuropathol Appl Neurobiol 2002;28:461–470.
21.
Confalonieri P, Oliva L, Andreetta F, Lorenzoni R, Dassi P, Mariani E, Morandi L, Mora M, Corelio F, Mantegazza R: Muscle inflammation and MHC class I up-regulation in muscular dystrophy with lack of dysferlin: an immunopathological study. J Neuroimmunol 2003;142:130–136.
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2006
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