Event Details:
Transmission of Misfolded Proteins in Neurodegenerative Disorders: A Common Mechanism of Disease Progression
Virginia Lee, Ph.D. Director - Center for Neurodegenerative Disease Research (CNDR), University of Pennsylvania
Host: Aaron Gitler
Abstract
The accumulation of misfolded proteins is a fundamental pathogenic process in neurodegenerative diseases. However, the factors that trigger aggregation and spreading of these disease proteins in brain are poorly understood. Recent studies demonstrate that misfolded disease proteins including alpha-synuclein in Parkinson’s disease and tau in Alzheimer’s disease and frontotemporal degeneration can be propagated from cell-to-cell through the recruitment of their endogenous normal counterparts. Moreover, pathologic misfolded aggregates propagated along major central nervous system (CNS) pathways to regions far beyond injection sites and appear to follow neuroanatomical interconnectomes. This spreading of pathology is progressive and leads to behavior impairments and eventually compromises neuronal survival but immune therapy reduces the spread of pathology, rescue behavior phenotypes and neuron loss. Our findings open up new avenues for understanding the mechanisms of disease progression and for developing novel therapeutics.