R-Loops in Motor Neuron Diseases.
Amyotrophic lateral sclerosis
DNA damage
Motor neuron disease
R-loops
Spinal muscular atrophy
Journal
Molecular neurobiology
ISSN: 1559-1182
Titre abrégé: Mol Neurobiol
Pays: United States
ID NLM: 8900963
Informations de publication
Date de publication:
Apr 2019
Apr 2019
Historique:
received:
29
04
2018
accepted:
15
07
2018
pubmed:
27
7
2018
medline:
7
8
2019
entrez:
27
7
2018
Statut:
ppublish
Résumé
R loops are transient three-stranded nucleic acid structures that form physiologically during transcription when a nascent RNA transcript hybridizes with the DNA template strand, leaving a single strand of displaced nontemplate DNA. However, aberrant persistence of R-loops can cause DNA damage by inducing genomic instability. Indeed, evidence has emerged that R-loops might represent a key element in the pathogenesis of human diseases, including cancer, neurodegeneration, and motor neuron disorders. Mutations in genes directly involved in R-loop biology, such as SETX (senataxin), or unstable DNA expansion eliciting R-loop generation, such as C9ORF72 HRE, can cause DNA damage and ultimately result in motor neuron cell death. In this review, we discuss current advancements in this field with a specific focus on motor neuron diseases associated with deregulation of R-loop structures. These mechanisms can represent novel therapeutic targets for these devastating, incurable diseases.
Identifiants
pubmed: 30047099
doi: 10.1007/s12035-018-1246-y
pii: 10.1007/s12035-018-1246-y
doi:
Substances chimiques
RNA-Binding Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Pagination
2579-2589Références
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