Aggresome Formation in Neuropathy Models Based on Peripheral Myelin Protein 22 Mutations

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...

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Bibliographic Details
Main Authors: Mary C. Ryan, Eric M. Shooter, Lucia Notterpek
Format: Article
Language:English
Published: Elsevier 2002-07-01
Series:Neurobiology of Disease
Subjects:
aggresomes
PMP22
ubiquitin-proteasome pathway
Trembler mice
protein degradation
Schwann cells
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996102905000

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http://www.sciencedirect.com/science/article/pii/S0969996102905000

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