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Division of Translational Neuromuscular Disease Research

The Division of Translational Neuromuscular Disease Research focuses its efforts in development of novel treatments for disorders of peripheral nerve, muscle, and neuromuscular transmission. The Division appreciates that only with detailed understanding of disease mechanisms can there be therapeutic advancement. Therefore, the Division also pursues studies basic to the understanding of nerve-muscle function and dysfunction.

Recruitment:
The Translational Neuromuscular Disease Division is actively seeking talented

  1. junior and mid-level faculty with establish research programs in the field of neuromuscular disease,
  2. post-doctoral fellows with experience in protein-based drug development, and
  3. research assistants with experience in skeletal muscle research.
    4. Please contact Henry J. Kaminski (hkaminsk@slu.edu)

Henry J. Kaminski, MD is the Director of the Division and Chairman of the Department of Neurology & Psychiatry. He has a long standing interest in extraocular muscle and their differential involvement by neuromuscular disease, and his research program has received continuous sponsorship by the National Institutes of Health since 1993. He has recently extended his work to development of complement inhibitor-based treatment of myasthenia gravis and muscular dystrophy. He continues to pursue work in understanding the cellular and molecular properties of EOM with the over-arching goal of manipulating EOM function for therapeutic endpoints. He is a member of the Executive Committee of a NIH-sponsored trial to evaluate the role of thymectomy in management of patients with myasthenia gravis and principal investigator of an ancillary study, Biomarkers in Myasthenia Gravis.


Extraocular Muscles in culture

Linda L. Kusner, PhD is an Assistant Professor in the Departments of Ophthalmology and Neurology & Psychiatry. Her investigations evaluate the extrinsic cues that foster the intrinsic programs that drive the unique EOM phenotype. These cellular responses are the result of distinct cell types that interact with the EOM at various stages of development as well as regeneration. The investigation into the unique nerve innervations of EOM has been well established. Research into the EOM’s connections to the obit that allow for eye movement is beginning to allow for greater understanding for strabismus. However, the environment of the EOM has not been evaluated with respect to fibroblast influences. The fibroblast may have a strong role in the development and regeneration of EOM. Dr. Kusner exploits an EOM specific cell line in concert with fibroblast co-cultures as a model system.

Jindrich Soltys, DVM, PhD obtained his degrees in Slovakia and continued in his postdoctoral training at Case Western Reserve University. He is currently Assistant Professor in the Department of Neurology & Psychiatry. He is involved in investigations to elucidate the pathogenesis of myasthenia gravis. Specifically, he is investigating the role of complement and its regulatory proteins on cellular immunity.


C9 depostion at neuromuscular junctions

Jiamin Teng, M.D., Ph.D. obtained his degrees in China and Japan and continued in his postdoctoral training in Louisiana State University Health Sciences Center. He is currently Assistant Professor in the Department of Neurology & Psychiatry. He is involved in studies concerning the extraocular muscle physiology and muscular reconstruction.

Yuefang Zhou, PhD received her doctoral degree from the Department of Neurosciences at Case Western Reserve University and then worked as a postdoctoral fellow before she joining the Division. She is currently is an Assistant Professor in the Department of Neurology & Psychiatry. Her primary research interest lies in how Pitx2, a homeobox transcription factor, regulates the postnatal growth and phenotypic maintenance of extraocular muscles and cardiac muscles. Preliminary studies have demonstrated that EOM contractility may be modified by alterations in Pitx2 expression, which has therapeutic implications.


The transcription factor Pitx2 is expressed by adult extraocular muscle.

Recent Publications
  1. Benatar M, Kaminski HJ. Evidence report: the medical treatment of ocular myasthenia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2007;68:2144-2149.
  2. Kaminski HJ. Restoring balance at the neuromuscular junction. Neurology 2007;69:629-630.
  3. Khanna S, Liao K, Kaminski HJ, Tomsak RL, Joshi A, Leigh RJ. Ocular myasthenia revisited: Insights from pseudo-internuclear ophthalmoplegia. J Neurol 2007;254:1569-1574.
  4. Zhou Y, Gong B, Lin F, Rother R, Medof ME, Kaminski HJ. Anti-C5 antibody treatment ameliorates weakness in experimentally acquired myasthenia gravis. J Immunol 2007;179:8562-67.
  5. Kusner L, Kaminski HJ, Soltys J. Effect of complement and its regulation on myasthenia gravis pathogenesis. Expert Rev Clin Immunol 2008;4:43-52.
  6. Luchanok U, Kaminski HJ. Ocular myasthenia: diagnostic and treatment recommendations and the evidence base. Curr Opin Neurol. 2008;21:8-15
  7. Zhou L, Rafael-Fortney JA, Huang P, Zhau XS, Cheng G, Zhou X, Kaminski HJ, Liu L, Ransohoff RM. Haploinsufficiency of utrophin gene worsens skeletal muscle inflammation and fibrosis in mdx mice. J Neurol Sci 2008;264:106-111.
  8. Newsom-Davis J, Cutter G, Wolfe GI, Kaminski HJ, Jaretzki A, Minisman G, Aban I, Conwit R. Status of the thymectomy trial for nonthymomatous myasthenia gravis patients receiving prednisone. Ann NY Acad Sci 2008;1132:344-347.
  9. Soltys J, Gong B, Kaminski HJ, Zhou Y, Kusner LL. Extaocular muscle susceptibility to myasthenia gravis. Unique Immunological Environment? Ann NY Acad Sci 2008:1132: 220-224.
  10. Cheng G, Kaminski HJ, Gong B, Zhou L, Hatala D, Howell S, Zhou X, Mustari M. Monocular deprivation in Macaque monkeys: A profile of gene expression in lateral geniculate nucleus by laser capture microdissection. Mol Vis 2008; 14:20-30.
  11. Aban I, Wolfe GI, Cutter GC, Kaminski HJ, Jaretzki A, Minisman G, Conwit R, Newsom-Davis J. The MGTX Experience: Challenges in Planning and Executing an International, Multicenter Clinical Trial, J Neuroimmunol 2008;201-2:80-84.
  12. Soltys J, Kusner LL, Young A, Richmonds C, Hatala D, Gong B, Shanmugavel V, Kaminski HJ. Novel Complement Inhibitor (rEV576) Limits Severity of Experimentally Acquired Myasthenia Gravis. Ann Neurol 2009;65:67-75.
  13. Alshekhlee A, Miles JD, Katirji B, Preston DC, Kaminski HJ. Incidence and Mortality Rates of Myasthenia Gravis and Myasthenic Crisis in U.S. Hospitals, Neurology 2009;72:1548–1554.
  14. Zhou Y, Cheng G, Dieter L, Hjalt TA, Andrade FH, Stahl JS, Kaminski HJ. An Altered Phenotype in a Conditional Knockout of Pitx2 in Extraocular Muscle. Invest Ophthalmol Vis Sci (in press).

http://www.youtube.com/watch?v=ZDNhsW_qMrw

http://www.fox2now.com/news/ktvi-foxfiles-tick-spit-050609,0,6384553.story

The Translational Neuromuscular Disease group is supported by an excellent Clinical Research Unit within the Department which can facilitate movement of technologies to Phase 1 trials. Allied clinical and basic departments offer a range of core facilities. Individuals interested in working with the Division of Translational Neuromuscular Disease should contact Henry J. Kaminski, M.D, Professor and Chairman, Department of Neurology & Psychiatry, Saint Louis University at hkaminsk@slu.edu.


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