We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
RNA Dysregulation in Amyotrophic Lateral Sclerosis.
- Authors
Butti, Zoe; Patten, Shunmoogum A.
- Abstract
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.
- Subjects
GENETICS of amyotrophic lateral sclerosis; NEURODEGENERATION; MOTOR neurons; RNA metabolism; RNA splicing
- Publication
Frontiers in Genetics, 2019, pN.PAG
- ISSN
1664-8021
- Publication type
Article
- DOI
10.3389/fgene.2018.00712