The mitochondrial-associated ER membrane (MAM) compartment and its dysregulation in amyotrophic lateral sclerosis (ALS)

Sonam Parakh, Julie D. Atkin*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)105-113
Number of pages9
JournalSeminars in Cell and Developmental Biology
Volume112
DOIs
Publication statusPublished - Apr 2021

Keywords

  • ALS
  • Lipid homeostasis
  • MAM dysfunction

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