[BIC-announce] FW: TODAY: Killam Lecture - Tuesday, Feb 8, 2011 - Dr. Steven Scherer - How do Connexin Mutations Cause Brain Diseases

[Jennifer Chew, Ms.]

PLEASE DISCARD IF THIS IS A DUPLICATE.  THANK YOU. Jennifer 
 

Jennifer Chew

McConnell Brain Imaging Centre

MNI - WB317

3801 University Street

Montreal, Qc  H3A 2B4

Telephone:  514-398-8554

Fax:  514-398-2975

 

 

________________________________

From: MNISTAFF - Montreal Neurological Institute Staff [mailto:MNISTAFF at LISTS.MCGILL.CA] On Behalf Of Grace Flynn, Ms.
Sent: Monday, February 07, 2011 4:40 PM
To: MNISTAFF at LISTS.MCGILL.CA
Subject: Tomorrow: Killam Lecture - Tuesday, Feb 8, 2011 - Dr. Steven Scherer



 

Speaker:         Steven Scherer, M.D., Ph.D., Professor of Neurology, The University of Pennsylvania School of Medicine 

 

Title:              How do Connexin Mutations Cause Brain Diseases

 

Date:              Tuesday, February 8, 2011

 

Time:             4:00 pm

 

Place:              de Grandpre Communications Centre, MNI

 

 

Steven S. Scherer is a Professor of Neurology at the University of Pennsylvania. He received his B.S. (1977), as well as his M.D. and Ph.D. (1985) from the University of Michigan. His Ph.D. advisor was Dr. Stephen S. Easter. He did an internship in internal medicine (1985-86), and a residency in neurology (1986-1989) at the Hospital of the University of Pennsylvania. He was a Charles A. Dana fellow at the University of Pennsylvania from 1989-1991, in the laboratory of Dr. John Kamholz. He joined the faculty in 1991, obtaining the rank of Professor in 2001. He served as Vice Chair for Research in the Department of Neurology. He has an author on nearly 110 original research papers and more than 50 reviews. 

 

Dr. Scherer's chief scientific interest is the pathogenesis of peripheral neuropathies. His interest in peripheral nerve began with his Ph.D. thesis, and brought him to Penn's Department of Neurology, which has a distinguished history in this area. In 1993, he collaborated with Dr. Kurt Fischbeck, then a colleague at Penn, in the discovery that mutations in the gene that encodes the gap junction protein connexin32 cause the X-linked form of Charcot-Marie-Tooth disease (the eponym for inherited neuropathies). Thus began one of the main lines of research in his laboratory - what are the functions of gap junctions in the myelin sheath, and how do mutations in the connexin genes disrupt these functions? Along with his colleagues and students, Dr. Scherer showed that there are functional gap junctions in the PNS myelin sheath, that many connexin32 mutants cause a loss of function, that oligodendrocytes and Schwann cells express other connexins that probably have overlapping functions, that the Cx47 mutants associated with Pelizaeus-Merzbacher-like disease cause loss of function, that oligodendryocytes and astrocytes are couple by two pairs of heterotypic channels - Cx47:Cx43 and Cx32:Cx30. 

 

The structure and function of the myelinated axon is the other main focus. Dr. Scherer and his colleagues and students have illuminated the "molecular architecture" of myelinated axons. The issues here are what molecules form the myelin sheath, and what are their functional roles? The emerging evidence indicates that molecular interactions between axons and myelinating glial cells cause regional specializations in axons that are required for saltatory conduction. Further, because demyelination disrupts these regional specializations, salutatory conduction fails. The goals of this work are to understand the molecular basis for conduction, and restore conduction in demyelinating diseases such as multiple sclerosis.

 

The diagnosis and treatment of peripheral neuropathies is Dr. Scherer's clinic interest.