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- W2076753211 abstract "Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease caused by the death of motor neurons. While the exact molecular and cellular basis for motor neuron death is not yet fully understood, the current conceptualization is that multiple aberrant biological processes contribute. Among these, one of the most compelling is based on alterations of RNA metabolism. In this review, we examine how the normal process of cellular response to stress leading to RNA stress granule formation might become pathological, resulting in the formation of stable protein aggregates. We discuss the emerging roles of post-translational modifications of RNA binding proteins in the genesis of these aggregates. We also review the contemporary literature regarding the potential role for more widespread alterations in RNA metabolism in ALS, including alterations in miRNA biogenesis, spliceosome integrity and RNA editing. A hypothesis is presented in which aberrant RNA processing, modulated through pathological stress granule formation as a reflection of either mutations within intrinsically disordered or prion-like domains of critical RNA binding proteins, or the post-translational modification of RNA binding proteins, contributes directly to motor neuron death." @default.
- W2076753211 created "2016-06-24" @default.
- W2076753211 creator A5040124007 @default.
- W2076753211 creator A5063677262 @default.
- W2076753211 creator A5077281610 @default.
- W2076753211 creator A5082361223 @default.
- W2076753211 creator A5087316938 @default.
- W2076753211 date "2014-02-20" @default.
- W2076753211 modified "2023-10-16" @default.
- W2076753211 title "RNA metabolism in ALS: When normal processes become pathological" @default.
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