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RNA Dysregulation in Amyotrophic Lateral Sclerosis.


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Butti, Zoe et Patten, Shunmoogum A. (2019). RNA Dysregulation in Amyotrophic Lateral Sclerosis. Frontiers in Genetics , vol. 9 , nº 712. pp. 1-18. DOI: 10.3389/fgene.2018.00712.

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Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease and is characterized by the degeneration of upper and lower motor neurons. It has become increasingly clear that RNA dysregulation is a key contributor to ALS pathogenesis. The major ALS genes SOD1, TARDBP, FUS, and C9orf72 are involved in aspects of RNA metabolism processes such as mRNA transcription, alternative splicing, RNA transport, mRNA stabilization, and miRNA biogenesis. In this review, we highlight the current understanding of RNA dysregulation in ALS pathogenesis involving these major ALS genes and discuss the potential of therapeutic strategies targeting disease RNAs for treating ALS.

Type de document: Article
Mots-clés libres: ALS (amyotrophic lateral sclerosis); C9orf72; FUS; RNA processing; RNAi (RNA interference); TDP-43; antisense oligonucleotide-drug conjugates
Centre: Centre INRS-Institut Armand Frappier
Date de dépôt: 06 août 2019 14:26
Dernière modification: 06 août 2019 14:26
URI: https://espace.inrs.ca/id/eprint/8090

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