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Steven S. Scherer

Ruth Wagner Van Meter and J. Ray Van Meter Professor of Neurology
Department: Neurology
Graduate Group Affiliations

Contact information
450 Stemmler Hall
Philadelphia, PA 19104-6077
Office: (215) 349-5313
Fax: (215) 573-4454
Education:
B.S.
University of Michigan, 1977.
Ph.D.
University of Michigan, 1985.
M.D.
University of Michigan, 1985.
Post-Graduate Training
Internship, Department of Internal Medicine, Univ. of Pennsylvania, 1985-1986.
Residency, Department of Neurology, Univ. of Pennsylvania, 1986-1989.
Charles A. Dana Fellowship in Neuroscience, Univ. of Pennsylvania, 1989-1991.
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Description of Research Expertise

RESEARCH INTERESTS
My main interest is the pathogenesis of demyelinating diseases, particularly those caused by mutations in the genes that encode the gap junction proteins Cx32 and Cx47. My current projects in my scientific laboratory relate to (1) the role of Cx32 in the pathogenesis of inherited demyelinating neuropathy, (2) how gap junctions between astrocytes and oligodendrocytes affect the structure/function of CNS myelin, (3) how the myelin sheath organizes the structure of the axon, and (4) animal models of inherited neuropathies. In the clinic, I also participate in a large effort in finding new genetic causes of inherited neuropathies.

Description of Clinical Expertise

My clinical work is focused on people who are known (or are suspected to have) a peripheral neuropathy. To diagnose neuropathy, I perform a comprehensive history and focused neurological examination, typically including clinical neurophysiology ("EMG"). This assessment enables me classify someone's neuropathy (examples - diabetic, inherited, chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barre syndrome (GBS), vasculitic, toxic, idiopathic, "small fiber"), and guides further testing. Establishing the correct diagnosis can lead to treatments for neuropathies caused by compression, inflammation, vitamin deficiencies, amyloid, or toxins. In addition, I work with people to reduce their neuropathic pain.

Description of Other Expertise

Clinical electrophysiology - EMG

Selected Publications

Scherer, S.S., K.A. Kleopa, and M.D. Benson : Peripheral Neuropathies. In: Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease, 5th edition. R.N. Rosenberg and J.M. Pascual (eds.). Elsevier (in press), 2015.

Fridman, V., B. Bundy, M.M. Reilly, D. Pareyson, C. Bacon, J. Burns, J. Day, S. Feely, R.S. Finkel, T. Grider, C. Kirk, D.N. Herrmann, M. Laurá, J. Li, T. Lloyd, C. Sumner, F. Muntoni, S. Ramchandren, R. Shy, C.E. Siskind, S. Yum, I. Moroni, E. Pagliano, S. Zuchner, S.S. Scherer, and M.E. Shy : CMT Subtypes and disease burden in patients enrolled in the INC natural history study (6601) from 2009-2013. J. Neurol. Neurosurg. Psychiat. (in press), 2015.

Baets, J., Duan, X., Wu, Y., Smith, G., Seeley, W., Mademan, I., McGrath, N.M., Beadell, N.C., Khoury, J., Botuyan, M.-V., Mer, G., Worrell, G.A., Hojo, K., Laura, M., Liu, Y.-T., Senderek, J.P., Weis, J., Van den Bergh, P., Merrill, S.L., Reilly, M.M., Houlden, H., Scherer, S.S., De Jonghe, P, Dyck, P. J., Klein, C.J. : Maintenance methyltransferase defect causes a spectrum of neurological diseases. Brain (in press), 2015.

Abrams, C.K., S.S. Scherer, R. Flores-Obando, S. Wong, E. Lamantea, L. Farina, V. Scaioli, D. Pareyson, E. Salsano: A new mutation in GJC2 associated with subclinical leukodystrophy. J. Neurology 261: 1929-1938, 2014.

King, C.H., E. Lancaster, D. Salomon, E. Peles, S.S. Scherer : Kv7.2 regulates the function of peripheral sensory neurons. J. Comp. Neurol. 522: 3262-3280, 2014.

Willison, H.J. and S.S. Scherer : Ranvier revisited: Novel nodal antigens stimulate interest in GBS pathogenesis. Neurology 83: 106-108, 2014.

Caramins, M., J.G. Colebatch, M.N. Bainbridge, S.S. Scherer, C.K. Abrams, E.L. Hackett, M.M. Freidin, S.N. Jhangiani, M. Wang, Y. Wu, D.M. Muzny, R. Lindeman, R.A. Gibbs. : Exome sequencing identification of a GJB1 missense mutation in a kindred with X-linked spinocerebellar ataxia (SCA-X1). Hum. Mol. Genet. 22: 4329-4338, 2013.

Niu, J., L. Ding, J. Li, H. Kim, J. Liu, H. Li, T. Badea, I.D. Duncan, Y.-J. Son, S.S. Scherer, W. Luo: Modality-based segregation of ascending somatosensory axons in the spinal cord dorsal column. J. Neurosci. 33: 17691-17709, 2013.

Fleisher, J., M. Richie, R. Price, S.S. Scherer, J. Dalmau, and E. Lancaster : Acquired neuromyotonia heralding recurrent thymoma in myasthenia gravis. Arch. Neurol. 69: 303-311, 2013.

Abrams, C.K. and S.S. Scherer : Gap junctions in inherited disorders of the central nervous system. Biochim. Biophys. Acta 1818: 2030-2047, 2012.

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Last updated: 01/02/2015
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