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.
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Authors | Mark S Forman, Virginia M-Y Lee, John Q Trojanowski |
Journal | Trends in neurosciences
(Trends Neurosci)
Vol. 26
Issue 8
Pg. 407-10
(Aug 2003)
ISSN: 0166-2236 [Print] England |
PMID | 12900170
(Publication Type: Journal Article, Review)
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Chemical References |
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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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