Skip to Main content Skip to Navigation
Journal articles

Endoplasmic reticulum dysfunction in neurological disease

Abstract : Endoplasmic reticulum (ER) dysfunction is important in the pathogenesis of many neurological diseases. In this review, we examine the evidence for ER dysfunction in a range of neurological conditions including cerebral ischaemia, sleep apnoea, Alzheimer's disease, multiple sclerosis, amyotrophic lateral sclerosis, the prion diseases and Familial Encephalopathy with Neuroserpin Inclusion Bodies (FENIB). Protein misfolding in the endoplasmic reticulum initiates a well-studied 'Unfolded Protein Response' in energy-starved neurones during stroke that is relevant to the toxicity of reperfusion. The toxic peptide Aβ induces 'ER stress' in Alzheimer's disease leading to activation of similar pathways, while the accumulation of polymeric neuroserpin in the neuronal ER triggers a poorly understood 'ER overload response'. In other neurological disorders such as Parkinson's and Huntington's diseases ER dysfunction is well recognised but the mechanisms for this are less clear. By targeting components of these signalling responses, it may prove possible to ameliorate their toxic effects and treat a range of neurodegenerative conditions.
Document type :
Journal articles
Complete list of metadatas

Cited literature [84 references]  Display  Hide  Download

https://www.hal.inserm.fr/inserm-01296824
Contributor : Benoit Roussel <>
Submitted on : Friday, April 1, 2016 - 4:03:59 PM
Last modification on : Tuesday, March 24, 2020 - 1:20:04 PM
Long-term archiving on: : Monday, November 14, 2016 - 12:40:56 PM

Files

THELANCETNEUROLOGY-D-12-00012R...
Files produced by the author(s)

  •  Roussel LancetNeuro2013.pdf    Embargoed until : jamais  Publisher files allowed on an open archive

Licence


Distributed under a Creative Commons Attribution 4.0 International License

Identifiers

Collections

Citation

Benoit D. Roussel, Antonina Kruppa, Elena Miranda, Damian Crowther, David Lomas, et al.. Endoplasmic reticulum dysfunction in neurological disease. The Lancet Neurology, Elsevier, 2013, 12 (1), pp.105 - 118. ⟨10.1016/S1474-4422(12)70238-7⟩. ⟨inserm-01296824⟩

Share

Metrics

Record views

550

Files downloads

2582