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'Unfolding' pathways in neurodegenerative disease.

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.
AuthorsMark S Forman, Virginia M-Y Lee, John Q Trojanowski
JournalTrends in neurosciences (Trends Neurosci) Vol. 26 Issue 8 Pg. 407-10 (Aug 2003) ISSN: 0166-2236 [Print] England
PMID12900170 (Publication Type: Journal Article, Review)
Chemical References
  • Membrane Proteins
Topics
  • Adaptation, Physiological
  • Alzheimer Disease (physiopathology)
  • Animals
  • Apoptosis
  • Endoplasmic Reticulum (chemistry, metabolism)
  • Humans
  • Membrane Proteins (physiology)
  • Mice
  • Neurodegenerative Diseases (physiopathology)
  • Parkinson Disease (physiopathology)
  • Pelizaeus-Merzbacher Disease (physiopathology)
  • Protein Conformation
  • Protein Folding
  • Stress, Physiological (physiopathology)

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