Abstract
The present paper reports complex immunological anomalies associated with motor end-plate disease (Med) in mice. Motor end-plate disease is a severe neuromuscular disorder which leads to death (around the 25th of life) in the Medj/Medj mutant, while the heterozygotes quickly recover from mild manifestations. Medj/Medj and Medj/ + mice share some of the immunological aberrations: reduced PFC response to SRBC in 14-16 day old mice, with reduced suppressor cell function and precocious maturation of the cytotoxic response to allogeneic cells in 21-23 day old mice. The diminished PFC response is corrected in adult Medj/ + mice but persists in the small group of Medj/Medj which escape death and which were studied between the 6th and 16th week of life. In addition, the thymus and spleen of Medj/Medj mice are greatly reduced in size, a symptom which appears with the onset of the clinical disease. Also, a reduction in the NK activity in the small group of older, surviving mice was noted. T and B lymphocyte proportions and the proliferative responses to T cell mitogens were not impaired in 14-16 day old mice. The role of these abnormalities in the pathogenesis of the disease is not known. Since some of these anomalies are shared by Medj/Medj and Medj/ +, the latter of which present no or mild and transient neurological manifestations, there is no clear link between the immunological and neuromuscular disorders.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Bach M. A., Phan-Dinh-Tuy F., Tournier E., Chatenoud L., Bach J. F., Martin C., Degos J. D. Deficit of suppressor T cells in active multiple sclerosis. Lancet. 1980 Dec 6;2(8206):1221–1223. doi: 10.1016/s0140-6736(80)92480-0. [DOI] [PubMed] [Google Scholar]
- Berrih S., Gaud C., Bach M. A., Le Brigand H., Binet J. P., Bach J. F. Evaluation of T cell subsets in myasthenia gravis using anti-T cell monoclonal antibodies. Clin Exp Immunol. 1981 Jul;45(1):1–8. [PMC free article] [PubMed] [Google Scholar]
- Calkins C. E., Stutman O. Changes in suppressor mechanisms during postnatal development in mice. J Exp Med. 1978 Jan 1;147(1):87–97. doi: 10.1084/jem.147.1.87. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Cunningham A. J., Szenberg A. Further improvements in the plaque technique for detecting single antibody-forming cells. Immunology. 1968 Apr;14(4):599–600. [PMC free article] [PubMed] [Google Scholar]
- Duchen L. W., Stefani E. Electrophysiological studies of neuromuscular transmission in hereditary 'motor end-plate disease' of the mouse. J Physiol. 1971 Jan;212(2):535–548. doi: 10.1113/jphysiol.1971.sp009340. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Fambrough D. M., Drachman D. B., Satyamurti S. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science. 1973 Oct 19;182(4109):293–295. doi: 10.1126/science.182.4109.293. [DOI] [PubMed] [Google Scholar]
- Karmali R. A., Horrobin D. F. Abnormalities of thymus growth in dystrophic mice. Nature. 1976 Oct 21;263(5579):684–685. doi: 10.1038/263684a0. [DOI] [PubMed] [Google Scholar]
- Lefkovits I. Induction of antibody-forming cell clones in microcultures. Eur J Immunol. 1972 Aug;2(4):360–366. doi: 10.1002/eji.1830020412. [DOI] [PubMed] [Google Scholar]
- Ludwig C. L., Kanellopoulos-Langevin C., Kim K. J., Mathieson B. J., Morse H. C., 3rd Immunologic function and cell surface antigen expression of lymphocytes of dystrophic mice. Cell Immunol. 1981 Mar 15;59(1):138–150. doi: 10.1016/0008-8749(81)90441-x. [DOI] [PubMed] [Google Scholar]
- Wu S., Bach F. H., Auerbach R. Cell-mediated immunity: differential maturation of mixed leukocyte reaction and cell-mediated lympholysis. J Exp Med. 1975 Nov 1;142(5):1301–1305. doi: 10.1084/jem.142.5.1301. [DOI] [PMC free article] [PubMed] [Google Scholar]
- de Kretser T. A., Livett B. G. Evidence of a thymic abnormality in murine muscular dystrophy. Nature. 1976 Oct 21;263(5579):682–684. doi: 10.1038/263682a0. [DOI] [PubMed] [Google Scholar]