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American Journal of Pathology, Vol 145, 766-770, Copyright © 1994 by American Society for Investigative Pathology

REGULAR ARTICLES

Myogenesis in thymic transplants in the severe combined immunodeficient mouse model of myasthenia gravis. Differentiation of thymic myoid cells into striated muscle cells

S Spuler, A Marx, T Kirchner, R Hohlfeld and H Wekerle
Department of Neurology, Klinikum Grossbadern, University of Munich, Germany.

Thymic myoid cells (TMCs) bearing acetylcholine receptors (AchR) on their surface have a central role in the concept of intrathymic autosensitization in the pathogenesis of myasthenia gravis. In a SCID mouse model of myasthenia gravis, solid pieces of thymuses with lymphofollicular hyperplasia were transplanted into SCID mice. The chimeric mice displayed long-term secretion of anti-AchR antibodies. Here, we traced the fate of TMCs contained in transplanted myasthenia gravis thymuses. Unexpectedly, the number of thymic TMCs in transplanted tissue was slightly higher than in untransplanted thymus. More strikingly, the transplanted TMCs were more highly differentiated than their nontransplanted counterparts. This was demonstrated by a more than 10-fold increase of AchR-main immunogenic region epitopes recognized by monoclonal antibody 198. Some TMCs had even differentiated into striated muscle cells. The abundance of AchRs in human thymic transplants in the SCID mouse model of myasthenia gravis may help to study the mechanisms of autosensitization against the AchR in vivo.


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A. MARX, H. K. MULLER-HERMELINK, and P. STROBEL
The Role of Thymomas in the Development of Myasthenia Gravis
Ann. N.Y. Acad. Sci., September 1, 2003; 998(1): 223 - 236.
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Copyright © 1994 by the American Society for Investigative Pathology.