Disruption of ER-mitochondria Signalling in Fronto-temporal Dementia and Related Amyotrophic Lateral Sclerosis

Overview

Journal Cell Death Dis

Specialties Cell Biology
General Medicine

Date 2018 Mar 2

PMID 29491392

Citations 42

Authors

Dawn H W Lau

Naomi Hartopp

Natalie J Welsh

Sarah Mueller

Elizabeth B Glennon

Gabor M Morotz

Ambra Annibali

Patricia Gomez-Suaga

Radu Stoica

Sebastien Paillusson

Christopher C J Miller

Affiliations

Soon will be listed here.

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.

Citing Articles

Structural and functional studies of the VAPB-PTPIP51 ER-mitochondria tethering proteins in neurodegenerative diseases.

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The Underestimated Role of Iron in Frontotemporal Dementia: A Narrative Review.

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Aberrant ER-mitochondria communication is a common pathomechanism in mitochondrial disease.

Morcillo P, Kabra K, Velasco K, Cordero H, Jennings S, Yun T Cell Death Dis. 2024; 15(6):405.

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Targeting mitochondrial Ca uptake for the treatment of amyotrophic lateral sclerosis.

Zhong R, Rua M, Wei-LaPierre L J Physiol. 2023; 602(8):1519-1549.

PMID: 38010626 PMC: 11032238. DOI: 10.1113/JP284143.

Mitochondria, a Key Target in Amyotrophic Lateral Sclerosis Pathogenesis.

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