The neuromuscular junction may be viewed as a model synapse, easily studied in isolation from other synapses. Moreover, its components, especially the nicotine acetylcholine receptor (AChR), can readily be purified or isolated from electric organs of electric fish. We have been studying the structure of functionally-important portions of the AChR. Much of this work involves the use of monoclonal antibodies (mAbs) directed against these portions of the molecule. Antibodies of this type in vivo produce altered neuromuscular transmission that is identical to what occurs in the human neuromuscular disease myasthenia gravis.

Publications:

Mihovilovic, M., Donnelly-Roberts, D., Richman, D.P. and Martinez-Carrion, M. Pathogenesis of hyperacute experimental autoimmune myasthenia gravis. J. Immunol. 152:5997-6002, 1994.

Richman, D.P., Ed. Myasthenia Gravis and Related Diseases: Disorders of the Neuromuscular Junction. New York Academy of Sciences (Annals V. 841), New York, 838 pp., 1998.

Fairclough, R.H., Twaddle, G.M., Gudipati, E., Richman, D.P., Burkwall, D.A., and Josephs R. Mapping the mAb 383C epitope to alpha(187-199) of the Torpedo acetylcholine receptor on the three dimensional model. J. Mol. Biol. 282:301-315, 1998.

Fairclough, R.H., Twaddle, G.M., Gudipati, E., Lin, M.Y. and Richman, D.P. Differential surface accessibility of alpha(187-199) in the Torpedo acetylcholine receptor alpha subunits. J. Mol. Biol. 282:317-330, 1998.

Maselli, R.A., Kong, D., Bowe, C., McDonald, C., Ellis, W., Agius, M.A., Gomez, C.M., Richman, D.P., and Wollmann, R.L. Presynaptic congenital myasthenic syndrome due to quantal release deficiency. Neurology 57: 279-289, 2001.

Richman, D.P. and Agius, M.A. Treatment of autoimmune myasthenia gravis. Neurology. (in press). Fairclough, R.H., Agius, M.A., Gudipati, E., Silvian, L., Hamaoka, B., Beltzer, C.C., Lin, M.Y., Chuang, A. and Richman, D.P. Agonist induced transitions of the acetylcholine receptor. Ann NY Acad Sci (in press).