New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective, early degeneration of motor neurons in the brain and spinal cord. Motor neurons have long axonal projections, which rely on the integrity of neuronal cytoskeleton and mitochondria to r...

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Autores principales:	Frances Theunissen, Phillip K. West, Samuel Brennan, Bojan Petrović, Kosar Hooshmand, P. Anthony Akkari, Matt Keon, Boris Guennewig
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Cytoskeleton Neurofilament Mitochondria Amyotrophic lateral sclerosis Neurodegeneration Axonal transport Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/eab41bb05cec4708b2b7573c126632c9
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spelling	oai:doaj.org-article:eab41bb05cec4708b2b7573c126632c92021-11-21T12:31:40ZNew perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis10.1186/s40035-021-00272-z2047-9158https://doaj.org/article/eab41bb05cec4708b2b7573c126632c92021-11-01T00:00:00Zhttps://doi.org/10.1186/s40035-021-00272-zhttps://doaj.org/toc/2047-9158Abstract Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective, early degeneration of motor neurons in the brain and spinal cord. Motor neurons have long axonal projections, which rely on the integrity of neuronal cytoskeleton and mitochondria to regulate energy requirements for maintaining axonal stability, anterograde and retrograde transport, and signaling between neurons. The formation of protein aggregates which contain cytoskeletal proteins, and mitochondrial dysfunction both have devastating effects on the function of neurons and are shared pathological features across several neurodegenerative conditions, including ALS, Alzheimer's disease, Parkinson's disease, Huntington’s disease and Charcot-Marie-Tooth disease. Furthermore, it is becoming increasingly clear that cytoskeletal integrity and mitochondrial function are intricately linked. Therefore, dysregulations of the cytoskeletal network and mitochondrial homeostasis and localization, may be common pathways in the initial steps of neurodegeneration. Here we review and discuss known contributors, including variants in genetic loci and aberrant protein activities, which modify cytoskeletal integrity, axonal transport and mitochondrial localization in ALS and have overlapping features with other neurodegenerative diseases. Additionally, we explore some emerging pathways that may contribute to this disruption in ALS.Frances TheunissenPhillip K. WestSamuel BrennanBojan PetrovićKosar HooshmandP. Anthony AkkariMatt KeonBoris GuennewigBMCarticleCytoskeletonNeurofilamentMitochondriaAmyotrophic lateral sclerosisNeurodegenerationAxonal transportNeurology. Diseases of the nervous systemRC346-429ENTranslational Neurodegeneration, Vol 10, Iss 1, Pp 1-16 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Cytoskeleton Neurofilament Mitochondria Amyotrophic lateral sclerosis Neurodegeneration Axonal transport Neurology. Diseases of the nervous system RC346-429
spellingShingle	Cytoskeleton Neurofilament Mitochondria Amyotrophic lateral sclerosis Neurodegeneration Axonal transport Neurology. Diseases of the nervous system RC346-429 Frances Theunissen Phillip K. West Samuel Brennan Bojan Petrović Kosar Hooshmand P. Anthony Akkari Matt Keon Boris Guennewig New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
description	Abstract Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective, early degeneration of motor neurons in the brain and spinal cord. Motor neurons have long axonal projections, which rely on the integrity of neuronal cytoskeleton and mitochondria to regulate energy requirements for maintaining axonal stability, anterograde and retrograde transport, and signaling between neurons. The formation of protein aggregates which contain cytoskeletal proteins, and mitochondrial dysfunction both have devastating effects on the function of neurons and are shared pathological features across several neurodegenerative conditions, including ALS, Alzheimer's disease, Parkinson's disease, Huntington’s disease and Charcot-Marie-Tooth disease. Furthermore, it is becoming increasingly clear that cytoskeletal integrity and mitochondrial function are intricately linked. Therefore, dysregulations of the cytoskeletal network and mitochondrial homeostasis and localization, may be common pathways in the initial steps of neurodegeneration. Here we review and discuss known contributors, including variants in genetic loci and aberrant protein activities, which modify cytoskeletal integrity, axonal transport and mitochondrial localization in ALS and have overlapping features with other neurodegenerative diseases. Additionally, we explore some emerging pathways that may contribute to this disruption in ALS.
format	article
author	Frances Theunissen Phillip K. West Samuel Brennan Bojan Petrović Kosar Hooshmand P. Anthony Akkari Matt Keon Boris Guennewig
author_facet	Frances Theunissen Phillip K. West Samuel Brennan Bojan Petrović Kosar Hooshmand P. Anthony Akkari Matt Keon Boris Guennewig
author_sort	Frances Theunissen
title	New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
title_short	New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
title_full	New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
title_fullStr	New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
title_full_unstemmed	New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
title_sort	new perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
publisher	BMC
publishDate	2021
url	https://doaj.org/article/eab41bb05cec4708b2b7573c126632c9
work_keys_str_mv	AT francestheunissen newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT phillipkwest newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT samuelbrennan newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT bojanpetrovic newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT kosarhooshmand newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT panthonyakkari newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT mattkeon newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis AT borisguennewig newperspectivesoncytoskeletaldysregulationandmitochondrialmislocalizationinamyotrophiclateralsclerosis
_version_	1718418979131752448

New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis

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