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Trends Neurosci. 2003 Aug;26(8):407-10.

'Unfolding' pathways in neurodegenerative disease.

Author information

1
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania, 3600 Spruce Street, Maloney Building, 3rd Floor, Philadelphia, PA 19104, USA. formanm@mail.med.upenn.edu

Abstract

The endoplasmic reticulum responds to stress by initiating a cascade of events known as the 'unfolded-protein response' (UPR). The accumulation of misfolded proteins in the leukodystrophy Pelizaeus-Merzbacher disease activates this stress response, resulting in apoptosis of oligodendrocytes. Although it remains uncertain whether the UPR plays a mechanistic role in prototypical neurodegenerative disorders such as Alzheimer's disease, this is plausible because misfolded proteins are directly implicated in the pathogenesis of these disorders.

PMID:
12900170
DOI:
10.1016/S0166-2236(03)00197-8
[Indexed for MEDLINE]

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