Amyotrophic lateral sclerosis and skeletal muscle

An update

Research output: Contribution to journalArticle

30 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is the most frequent adult-onset motor neuron disease characterized by degeneration of upper and lower motor neurons (MNs), generalized weakness and muscle atrophy. The "neurocentric" view of ALS assumes that the disease primarily affects motor neurons, while muscle alterations only represent a consequence, in the periphery, of motor neuron loss. However, this outlook was recently challenged by evidence suggesting that non-neural cells such as microglia, astrocytes, peripheral blood mononuclear cells (PBMCs) and skeletal muscle fibres participate in triggering motor neuron degeneration, and this stressed the concept that alterations in different cell types may act together to exacerbate the disease. In this review, we will summarize the most recent findings on the alterations of skeletal muscle fibres found in ALS, with particular attention to the relationship between mutant SOD1 and skeletal muscle. We will analyze changes in muscle function, in the expression of myogenic regulatory factors, and also mitochondrial dysfunction, SOD1 aggregation and proteasome activity.

Original languageEnglish
Pages (from-to)984-990
Number of pages7
JournalMolecular Neurobiology
Volume49
Issue number2
DOIs
Publication statusPublished - 2014

Fingerprint

Amyotrophic Lateral Sclerosis
Motor Neurons
Skeletal Muscle
Skeletal Muscle Fibers
Myogenic Regulatory Factors
Muscles
Nerve Degeneration
Motor Neuron Disease
Muscular Atrophy
Microglia
Proteasome Endopeptidase Complex
Astrocytes
Blood Cells

Keywords

  • Amyotrophic lateral sclerosis
  • Mitochondria
  • Myogenic factors
  • Proteasome
  • Skeletal muscle fibres

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience
  • Medicine(all)

Cite this

Amyotrophic lateral sclerosis and skeletal muscle : An update. / Pansarasa, O.; Rossi, D.; Berardinelli, A.; Cereda, C.

In: Molecular Neurobiology, Vol. 49, No. 2, 2014, p. 984-990.

Research output: Contribution to journalArticle

@article{aefc3308bcd54aedb479116a98c16d3e,
title = "Amyotrophic lateral sclerosis and skeletal muscle: An update",
abstract = "Amyotrophic lateral sclerosis (ALS) is the most frequent adult-onset motor neuron disease characterized by degeneration of upper and lower motor neurons (MNs), generalized weakness and muscle atrophy. The {"}neurocentric{"} view of ALS assumes that the disease primarily affects motor neurons, while muscle alterations only represent a consequence, in the periphery, of motor neuron loss. However, this outlook was recently challenged by evidence suggesting that non-neural cells such as microglia, astrocytes, peripheral blood mononuclear cells (PBMCs) and skeletal muscle fibres participate in triggering motor neuron degeneration, and this stressed the concept that alterations in different cell types may act together to exacerbate the disease. In this review, we will summarize the most recent findings on the alterations of skeletal muscle fibres found in ALS, with particular attention to the relationship between mutant SOD1 and skeletal muscle. We will analyze changes in muscle function, in the expression of myogenic regulatory factors, and also mitochondrial dysfunction, SOD1 aggregation and proteasome activity.",
keywords = "Amyotrophic lateral sclerosis, Mitochondria, Myogenic factors, Proteasome, Skeletal muscle fibres",
author = "O. Pansarasa and D. Rossi and A. Berardinelli and C. Cereda",
year = "2014",
doi = "10.1007/s12035-013-8578-4",
language = "English",
volume = "49",
pages = "984--990",
journal = "Molecular Neurobiology",
issn = "0893-7648",
publisher = "Humana Press Inc.",
number = "2",

}

TY - JOUR

T1 - Amyotrophic lateral sclerosis and skeletal muscle

T2 - An update

AU - Pansarasa, O.

AU - Rossi, D.

AU - Berardinelli, A.

AU - Cereda, C.

PY - 2014

Y1 - 2014

N2 - Amyotrophic lateral sclerosis (ALS) is the most frequent adult-onset motor neuron disease characterized by degeneration of upper and lower motor neurons (MNs), generalized weakness and muscle atrophy. The "neurocentric" view of ALS assumes that the disease primarily affects motor neurons, while muscle alterations only represent a consequence, in the periphery, of motor neuron loss. However, this outlook was recently challenged by evidence suggesting that non-neural cells such as microglia, astrocytes, peripheral blood mononuclear cells (PBMCs) and skeletal muscle fibres participate in triggering motor neuron degeneration, and this stressed the concept that alterations in different cell types may act together to exacerbate the disease. In this review, we will summarize the most recent findings on the alterations of skeletal muscle fibres found in ALS, with particular attention to the relationship between mutant SOD1 and skeletal muscle. We will analyze changes in muscle function, in the expression of myogenic regulatory factors, and also mitochondrial dysfunction, SOD1 aggregation and proteasome activity.

AB - Amyotrophic lateral sclerosis (ALS) is the most frequent adult-onset motor neuron disease characterized by degeneration of upper and lower motor neurons (MNs), generalized weakness and muscle atrophy. The "neurocentric" view of ALS assumes that the disease primarily affects motor neurons, while muscle alterations only represent a consequence, in the periphery, of motor neuron loss. However, this outlook was recently challenged by evidence suggesting that non-neural cells such as microglia, astrocytes, peripheral blood mononuclear cells (PBMCs) and skeletal muscle fibres participate in triggering motor neuron degeneration, and this stressed the concept that alterations in different cell types may act together to exacerbate the disease. In this review, we will summarize the most recent findings on the alterations of skeletal muscle fibres found in ALS, with particular attention to the relationship between mutant SOD1 and skeletal muscle. We will analyze changes in muscle function, in the expression of myogenic regulatory factors, and also mitochondrial dysfunction, SOD1 aggregation and proteasome activity.

KW - Amyotrophic lateral sclerosis

KW - Mitochondria

KW - Myogenic factors

KW - Proteasome

KW - Skeletal muscle fibres

UR - http://www.scopus.com/inward/record.url?scp=84896543905&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84896543905&partnerID=8YFLogxK

U2 - 10.1007/s12035-013-8578-4

DO - 10.1007/s12035-013-8578-4

M3 - Article

VL - 49

SP - 984

EP - 990

JO - Molecular Neurobiology

JF - Molecular Neurobiology

SN - 0893-7648

IS - 2

ER -