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Disruption of ER-mitochondria signalling in fronto-temporal dementia and related amyotrophic lateral sclerosis

Lau, DHW; Hartopp, N; Welsh, NJ; Mueller, S; Glennon, EB; Morotz, GM; Annibali, A; ... Miller, CCJ; + view all (2018) Disruption of ER-mitochondria signalling in fronto-temporal dementia and related amyotrophic lateral sclerosis. Cell Death & Disease , 9 , Article 327. 10.1038/s41419-017-0022-7. Green open access

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Abstract

Fronto-temporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are two related and incurable neurodegenerative diseases. Features of these diseases include pathological protein inclusions in affected neurons with TAR DNA-binding protein 43 (TDP-43), dipeptide repeat proteins derived from the C9ORF72 gene, and fused in sarcoma (FUS) representing major constituent proteins in these inclusions. Mutations in C9ORF72 and the genes encoding TDP- 43 and FUS cause familial forms of FTD/ALS which provides evidence to link the pathology and genetics of these diseases. A large number of seemingly disparate physiological functions are damaged in FTD/ALS. However, many of these damaged functions are regulated by signalling between the endoplasmic reticulum and mitochondria, and this has stimulated investigations into the role of endoplasmic reticulum-mitochondria signalling in FTD/ALS disease processes. Here, we review progress on this topic.

Type: Article
Title: Disruption of ER-mitochondria signalling in fronto-temporal dementia and related amyotrophic lateral sclerosis
Open access status: An open access version is available from UCL Discovery
DOI: 10.1038/s41419-017-0022-7
Publisher version: http://dx.doi.org/10.1038/s41419-017-0022-7
Language: English
Additional information: © The Author(s) 2018 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
Keywords: Science & Technology, Life Sciences & Biomedicine, Cell Biology, C9ORF72 HEXANUCLEOTIDE REPEAT, PLURIPOTENT STEM-CELLS, ENDOPLASMIC-RETICULUM, SIGMA-1 RECEPTOR, NEURODEGENERATIVE DISEASES, REGULATES AUTOPHAGY, CONTACT SITES, MOUSE MODEL, COGNITIVE IMPAIRMENT, LOBAR DEGENERATION
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > UK Dementia Research Institute
URI: https://discovery.ucl.ac.uk/id/eprint/10046623
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