[BIC-announce] FW: Killam Lecture - Tuesday, February 28, 2006 The role of the cytoskeleton in neurodegenerative diseases

Mon Feb 27 09:42:43 EST 2006

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 Enza Ferracane, Ms.
Sent: Monday, February 27, 2006 9:15 AM
To: MNISTAFF at LISTS.MCGILL.CA
Subject: Killam Lecture - Tuesday, February 28, 2006

KILLAM LECTURE

Date:  Tuesday, February 28, 2006 - TOMORROW

Speaker:  Ron Liem, Ph.D.

Professor, Columbia University

Title:  The Role of the Cytoskeleton in Neurodegenerative Diseases

Place:  de Grandpre Communications Centre

Time:  4:00 pm 
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		Ronald Liem, Ph.D.

Professor

E-mail: rkl2 at columbia.edu 

Cellular and molecular studies of the neuronal cytoskeleton

Our laboratory studies the neuronal cytoskeleton and its relationship to
neurodegenerative diseases. In the mammalian nervous system a variety of
neuronal intermediate filament proteins are expressed. Overexpression
and mutations of the neuronal intermediate filament proteins leads to
neuronal degeneration, showing that malfunctions in neuronal
intermediate filaments can be responsible for neurodegenerative
diseases. Recent studies have shown that neurofilaments are mutated in
Charcot-Marie-Tooth disease, an inherited neurodegenerative disease. We
are studying the effects that these mutations have on neurofilament
assembly and transport to try and understand how these mutations lead to
neurodegeneration. We are also studying the microtubule associated
protein tau. One of the hallmarks of Alzheimer's Disease is the presence
of paired helical filaments, which are composed of tau. Recent studies
have identified mutations in tau in a hereditary neurodegenerative
disease, frontotemporal Dementia with Parkinsonism. By using transgenic
models, we hope to determine how these mutations might lead to
neurodegeneration 

In recent studies, we have also focused on a family of cytoskeletal
linker proteins called plakins. These proteins connect cytoskeletal
elements to each other and to junctional complexes. They consist of
combinations of interacting domains, which include domains that bind to
microfilaments, microtubules, intermediate filaments, cell adhesion
molecules, as well as members of the catenin family. Mutations in these
proteins lead to a variety of cellular defects, including skin
blistering, muscular dystrophy and axonal outgrowth defects. Two
proteins, microtubule actin crosslinking factor (MACF) and Bullous
Pemphiogid Antigen 1 (BPAG1) are expressed in the muscle and the nervous
system. The neuronal isosforms of these proteins are involved in axon
outgrowth. We are identifying their interactions partners and and we are
studying the functions of these proteins in vivo. 

Selected publications

Leung C.L., Zheng M., Prater S,M and Liem R.K.H. The BPAG1 locus:
alternative splicing produces multiple isoforms with distinct
cytoskeletal linker domains, including predominant isoforms in neurons
and muscles. J. Cell Biol. In press. 

Sun, D., Leung, C.L. and Liem, R.K.H. 2001. Characterization of the
microtubule binding domain of Microtubule Actin Crosslinking Factor
(MACF): identification of a novel group of microtubule associated
proteins. J. Cell. Sci.114:161-172. 

Wang, L., Ho, C.-L., Sun, D., Liem, R.K.H. and Brown, 2000. A. Rapid
movement of axonal neurofilaments interrupted by prolonged pauses.
Nature Cell Biol. 2:137-141. 

Leung, C.L., Sun, D., Zheng, M., Knowles, D.R. and Liem, R.K.H. 1999.
Microtubule Actin Cross-Linking Factor (MACF): A hybrid of dystonin and
dystrophin that can interact with the actin and microtubule
cytoskeletons. J. Cell Biol. 147:1275-1285. 

Ching, G.Y., Chien, C.-L., Flores, R. G and Liem, R.K.H. 1999.
Overexpression of a-internexin causes abnormal neurofilamentous
accumulations and motor coordination deficits in transgenic mice. J.
Neurosci.19:2974-2986. 

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-- 

Wayne Sossin 

Associate Professor, McGill University 

Dept. Neurology and Neurosurgery 

Dept. Anatomy and Cell Biology 

3801 University St. 

Montreal Neurological Institute 

Montreal, Quebec 

Canada H3A-2B4 

Phone: 1-514-398-1486 

Fax: 1-514-398-8106 

e-mail wayne.sossin at mcgill.ca 

web-page http://www.mcgill.ca/mni/cbet/waynes/index.html 

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