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Transcript
Neuroscience Center of Excellence
School of Medicine
Seminar in Neuroscience
Why Corticospinal Motor
Neurons Are Important For
ALS and Other Motor
Neuron Diseases
Founding Director of ALS
Research Laboratory II
at Northwestern University,
Assistant Professor,
Ken and Ruth Davee
Department of Neurology
Feinberg School of Medicine,
Northwestern University,
Chicago, Illinois
12:00 p.m.
Monday
February 16, 2015
8th Floor
Neuroscience Center
of Excellence
Conference Room
more info: [email protected]
layout&design e.d. guillot NCE 020915
Pembe Hande Ozdinler, Ph.D.
Corticospinal motor neurons (CSMN) are some of the
most important cortical components of motor neuron
circuitry. Their unique ability to collect, integrate,
translate and transmit the brain's input to the spinal cord
targets allow them to function as the spokesperson for
the cerebral cortex for the initiation and modulation of
voluntary movement. CSMN vulnerability and progressive
degeneration is key in numerous motor neuron diseases,
such as primary lateral scalerosis, hereditary spastic
paraplegia, and amyotrophic lateral sclerosis. CSMN
death also leads to long-term paralysis in spinal cord
injury patients. Therefore, it is important to understand
the cellular and molecular mechanisms that are
responsible for the vulnerability and degeneration of this
clinically-relevant neuron population. Since their numbers
are very limited and there are no cellular markers to label
them, investigation of CSMN biology has been
challenging. We developed novel tools and approaches to
make these neurons visible in vivo, and characterized a
model system in which CSMN display progressive
degeneration. Recently we identified ER-stress to be one
of the underlying cellular mechanisms that leads to their
degeneration, which becomes evident by apical dendrite
degeneration and spine loss. Understanding the biology
behind CSMN degeneration will help develop effective
treatment strategies for many motor neuron diseases in
which voluntary movement is impaired.