期刊
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
卷 112, 期 -, 页码 105-113出版社
ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
DOI: 10.1016/j.semcdb.2021.02.002
关键词
ALS; MAM dysfunction; Lipid homeostasis
资金
- National Health and Medical Research Council of Australia (NHMRC) [1006141, 10305133, 1086887, 1095215]
- Motor Neurone Disease Research Institute of Australia
- Angie Cunningham Laugh to Cure MND Grant
- Zo-ee Research Grant
- FightMND Foundation
MAMs, formed at the junctions of the endoplasmic reticulum (ER) and mitochondria, play crucial roles in regulating cellular functions, homeostasis, and survival. Dysregulation of MAMs is implicated in various human diseases, including neurodegenerative diseases like ALS. Dysfunction of ER and mitochondria, as well as alterations in lipid metabolism in neurons, have been well documented in ALS, highlighting the potential therapeutic benefits of targeting MAM in this disorder.
The endoplasmic reticulum (ER) and mitochondria connect at multiple contact sites to form a unique cellular compartment, termed the ?mitochondria-associated ER membranes? (MAMs). MAMs are hubs for signalling pathways that regulate cellular homeostasis and survival, metabolism, and sensitivity to apoptosis. MAMs are therefore involved in vital cellular functions, but they are dysregulated in several human diseases. Whilst MAM dysfunction is increasingly implicated in the pathogenesis of neurodegenerative diseases, its role in amyotrophic lateral sclerosis (ALS) is poorly understood. However, in ALS both ER and mitochondrial dysfunction are well documented pathophysiological events. Moreover, alterations to lipid metabolism in neurons regulate processes linked to neurodegenerative diseases, and a link between dysfunction of lipid metabolism and ALS has also been proposed. In this review we discuss the structural and functional relevance of MAMs in ALS and how targeting MAM could be therapeutically beneficial in this disorder.
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