PMP22 Accumulation in Aggresomes: Implications for CMT1A Pathology

Peripheral myelin protein 22 (PMP22) is a 22-kDa glycoprotein mainly expressed by Schwann cells (SCs). Duplication or deletion of the PMP22 gene locus is associated with heritable peripheral neuropathies suggesting that the correct level of PMP22 protein is essential for SC functioning. Previously w...

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Bibliographic Details
Main Authors: Lucia Notterpek, Mary C. Ryan, Andreas R. Tobler, Eric M. Shooter
Format: Article
Language:English
Published: Elsevier 1999-10-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996199902747