Lucia Notterpek Lucia Notterpek investigates the role of peripheral myelin protein 22 in peripheral nerve biology, and the disease mechanisms of inherited demyelinating neuropathies, such as protein aggregation and protein degradation. Associate Professor of Neuroscience Investigator, McKnight Brain Institute Training Postdoc - Department of Neurobiology, Stanford University, CA, Mentor: Eric M. Shooter Ph.D. Neuroscience, UCLA, University of California, Los Angeles, CA B.S. Physiology and Anatomy, University of California at Berkeley, Berkeley, CA Contact phone: 352.294.0030 office: MBI L1-100E lab: MBI L2-164 email: notterp@mbi.ufl.edu The majority of inherited demyelinating peripheral neuropathies are linked to misexpression of peripheral myelin protein 22 (PMP22), yet the function of the protein in peripheral nerve biology is unclear. The primary goal of my research is to elucidate the disease mechanism of PMP22-associated neuropathies, with a focus on the role of protein mistrafficking in this process. Utilizing a variety of in vitro and in vivo approaches we found that disease-linked forms of PMP22 accumulate in cytosolic aggregates. The formation of protein aggregates in Schwann cells might represent a protective mechanism since under permissive condition the aggregates can be cleared. Now we are investigating how the presence and clearance of aggregated proteins affect normal Schwann cell biology. Our second major goal is to elucidate the role of PMP22 in normal peripheral nerve development, including Schwann cell myelination. Recent Publications Impaired proteasome activity and accumulation of ubiquitinated substrates in a hereditary neuropathy model. Fortun J, Li J, Go J, Fenstermaker A, Fletcher BS, Notterpek L. J Neurochem. 2005 Mar;92(6):1531-41. Erratum in: J Neurochem. 2005 May;93(3):766-8. Modulation of epithelial morphology, monolayer permeability, and cell migration by growth arrest specific 3/peripheral myelin protein 22. Roux KJ, Amici SA, Fletcher BS, Notterpek L. Mol Biol Cell. 2005 Mar;16(3):1142-51. Epub 2005 Jan 5. The protooncogene Ski controls Schwann cell proliferation and myelination. Atanasoski S, Notterpek L, Lee HY, Castagner F, Young P, Ehrengruber MU, Meijer D, Sommer L, Stavnezer E, Colmenares C, Suter U. Neuron. 2004 Aug 19;43(4):499-511.