The Cellular and Molecular Signature of ALS in Muscle.
motor neurone disease
muscle metabolism
muscle regeneration
muscle secretome
muscle-nerve communication
neuromuscular junction
Journal
Journal of personalized medicine
ISSN: 2075-4426
Titre abrégé: J Pers Med
Pays: Switzerland
ID NLM: 101602269
Informations de publication
Date de publication:
08 Nov 2022
08 Nov 2022
Historique:
received:
29
09
2022
revised:
29
10
2022
accepted:
01
11
2022
entrez:
29
12
2022
pubmed:
30
12
2022
medline:
30
12
2022
Statut:
epublish
Résumé
Amyotrophic lateral sclerosis is a disease affecting upper and lower motor neurons. Although motor neuron death is the core event of ALS pathology, it is increasingly recognized that other tissues and cell types are affected in the disease, making potentially major contributions to the occurrence and progression of pathology. We review here the known cellular and molecular characteristics of muscle tissue affected by ALS. Evidence of toxicity in skeletal muscle tissue is considered, including metabolic dysfunctions, impaired proteostasis, and deficits in muscle regeneration and RNA metabolism. The role of muscle as a secretory organ, and effects on the skeletal muscle secretome are also covered, including the increase in secretion of toxic factors or decrease in essential factors that have consequences for neuronal function and survival.
Identifiants
pubmed: 36579600
pii: jpm12111868
doi: 10.3390/jpm12111868
pmc: PMC9692882
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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