Description:

Anne Schaefer, MD, PhD
Assistant Professor
Friedman Brain Institute
Icahn School of Medicine at Mount Sinai
Department of Neuroscience and Psychiatry
New York, NY

Understanding the mechanisms that protect neurons against degeneration is significant for the prevention and treatment of neurodegenerative diseases in humans. We found that Polycomb repressive complex 2 (PRC2) that contributes to neuronal differentiation during development protects neurons against neurodegeneration. Here we show that PRC2 couples the maintenance of neuron identity to neuronal survival in the adult brain. Deficiency of PRC2 in neurons abolishes their functional specification and leads to the activation of PRC2 controlled death-inducing genes. These changes in neuronal cell identity and survival lead to the development of fatal Huntington's-like neurodegenerative disorders in mice. Our data suggests that abnormal function of the PRC2 and related epigenetic processes can represent a common mechanism for progressive neurodegeneration in mice and humans.