ALS: Lou Gehrig’s Disease

Cortical mechanisms in a mouse model of amyotrophic lateral sclerosis
2011 Seed Grant
Gordon Shepherd, M.D., Ph.D.
Department of Physiology
Northwestern University

The neurodegenerative disease amyotrophic lateral sclerosis (ALS; Lou Gehrig’s disease) is a fatal paralytic disorder characterized by cell death of corticospinal neurons. Early in the disease process cortical circuits are hyperexcitable, but specific mechanisms have not been identified. High-resolution imaging and electrophysiological analysis of specific microcircuits of corticospinal neurons will reveal, for the first time, how these neurons are ‘mis-wired’ in ALS, with high potential for identifying new cellular/molecular targets for therapeutic interventions.

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