Endoplasmic reticulum and mitochondria in diseases of motor and sensory neurons: a broken relationship?

Cell Death & Disease
Nathalie Bernard-MarissalBernard L Schneider

Abstract

Recent progress in the understanding of neurodegenerative diseases revealed that multiple molecular mechanisms contribute to pathological changes in neurons. A large fraction of these alterations can be linked to dysfunction in the endoplasmic reticulum (ER) and mitochondria, affecting metabolism and secretion of lipids and proteins, calcium homeostasis, and energy production. Remarkably, these organelles are interacting with each other at specialized domains on the ER called mitochondria-associated membranes (MAMs). These membrane structures rely on the interaction of several complexes of proteins localized either at the mitochondria or at the ER interface and serve as an exchange platform of calcium, metabolites, and lipids, which are critical for the function of both organelles. In addition, recent evidence indicates that MAMs also play a role in the control of mitochondria dynamics and autophagy. MAMs thus start to emerge as a key element connecting many changes observed in neurodegenerative diseases. This review will focus on the role of MAMs in amyotrophic lateral sclerosis (ALS) and hereditary motor and sensory neuropathy, two neurodegenerative diseases particularly affecting neurons with long projecting axons. We will d...Continue Reading

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Citations

Mar 20, 2018·Cell Death & Disease·Paolo Pinton
Apr 6, 2018·Molecular Neurobiology·Juan A GodoyNibaldo C Inestrosa
Jan 20, 2019·Proceedings of the National Academy of Sciences of the United States of America·Nathalie Bernard-MarissalRoman Chrast
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Aug 28, 2021·Cells·Nica BorgeseFrancesca Navone

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Methods Mentioned

BETA
GTPases
GTPase
electron microscopy
protein folding
ubiquitination

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