Aggresome Formation in Neuropathy Models Based on Peripheral Myelin Protein 22 Mutations
Alterations in peripheral myelin protein 22 (PMP22) gene expression are associated with demyelinating peripheral neuropathies. Overexpression of wild type (wt) PMP22 or inhibition of proteasomal degradation lead to the formation of aggresomes, intracellular ubiquitinated PMP22 aggregates. Aggresome...
Main Authors: | Mary C. Ryan, Eric M. Shooter, Lucia Notterpek |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2002-07-01
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Series: | Neurobiology of Disease |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996102905000 |
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