Lucia Notterpek, Ph.D.

Lucia NotterpekChair and Professor

Department of Neuroscience
University of Florida
1149 Newell Drive
PO Box 100244
Gainesville, FL  32610-0244
Office Phone: (352) 294-5373
Lab Phone: (352) 273-7323
Lab Members: Notterpek lab


1994 Ph.D.
University of California
Los Angeles, CA
1994-1999 Postdoctoral Fellow
Stanford University
Stanford, CA

Key Words: Peripheral neuropathies, Schwann cells, myelin, demyelinating disorders, autophagy, chaperones

Research Focus & Aims:

The primary goal of my research is to contribute toward the development of effective therapies for demyelinating disorders of the nervous system, such as hereditary neuropathies and multiple sclerosis. To accomplish this aim, we must understand the subcellular mechanisms that underlie the process of myelination during development and the maintenance of the myelin sheath in the mature nervous system. Approaches used include small molecule therapies and in vitro cellular modeling of demyelinating disorders.

The goal of our research is to understand how misregulation of glial gene expression contributes to peripheral neuropathies and other neurodegenerative processes within the nervous system. Specifically, ongoing investigations address how protein homeostatic mechanisms, including the chaperone and autophagy-lysosomal pathways, are involved in the subcellular pathogenesis of demyelinating neuropathies and in the age-related decline of neural function. While we investigate the contribution of these basic subcellular mechanisms to disease processes, we are also exploring dietary and pharmacologic approaches to modulate these pathways and keep them functional during aging. The main model system we use for our studies is Charcot-Marie-Tooth disease type 1A, an autosomal dominant demyelinating disorder of the peripheral nervous system. Using optical imaging we are examining the response of myelinating cells to the accumulation of damaged and misfolded proteins and investigating approaches to prevent and reverse these events. Therapeutic approaches under investigations include small molecule enhancers of protein degradation and protein folding, as well as miRNA-mediated gene suppression. Targeting of therapeutic and imaging molecules to specific cells is being achieved by the use of lipid nanoparticles.

Members of the Notterpek Lab — July 2017

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