Translation dysregulation in neurodegenerative diseases: a focus on ALS

RNA translation is tightly controlled in eukaryotic cells to regulate gene expression and maintain proteome homeostasis. RNA binding proteins, translation factors, and cell signaling pathways all modulate the translation process. Defective translation is involved in multiple neurological diseases in...

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Bibliographic Details
Published in:Molecular neurodegeneration 2023-08, Vol.18 (1), p.58-58, Article 58
Main Authors: Wang, Shaopeng, Sun, Shuying
Format: Article
Language:English
Subjects:
ALS
Alzheimer's disease
Amino acids
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - genetics
Binding proteins
Cell signaling
Cellular signal transduction
Degeneration
Development and progression
Enzymes
Gene expression
Gene mutations
Genetic translation
Homeostasis
Humans
Kinases
Metabolism
Nerve Degeneration
Nervous system
Neurodegeneration
Neurodegenerative diseases
Neurodegenerative Diseases - genetics
Neurological diseases
Oxidative stress
Peptides
Phosphorylation
Physiological aspects
Protein binding
Protein synthesis
Proteins
Proteomes
Public health
Review
Ribonucleic acid
RNA
RNA binding protein
Transfer RNA
Translation
Translation elongation
Translation initiation
Translation regulation
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Summary:RNA translation is tightly controlled in eukaryotic cells to regulate gene expression and maintain proteome homeostasis. RNA binding proteins, translation factors, and cell signaling pathways all modulate the translation process. Defective translation is involved in multiple neurological diseases including amyotrophic lateral sclerosis (ALS). ALS is a progressive neurodegenerative disorder and poses a major public health challenge worldwide. Over the past few years, tremendous advances have been made in the understanding of the genetics and pathogenesis of ALS. Dysfunction of RNA metabolisms, including RNA translation, has been closely associated with ALS. Here, we first introduce the general mechanisms of translational regulation under physiological and stress conditions and review well-known examples of translation defects in neurodegenerative diseases. We then focus on ALS-linked genes and discuss the recent progress on how translation is affected by various mutant genes and the repeat expansion-mediated non-canonical translation in ALS.
ISSN:1750-1326
1750-1326
DOI:10.1186/s13024-023-00642-3