RNA Degradation in Neurodegenerative Disease
- 19 June 2018
- book chapter
- Published by Springer Nature
- Vol. 20, 103-142
Abstract
Ribonucleic acid (RNA) homeostasis is dynamically modulated in response to changing physiological conditions. Tight regulation of RNA abundance through both transcription and degradation determines the amount, timing, and location of protein translation. This balance is of particular importance in neurons, which are among the most metabolically active and morphologically complex cells in the body. As a result, any disruptions in RNA degradation can have dramatic consequences for neuronal health. In this chapter, we will first discuss mechanisms of RNA stabilization and decay. We will then explore how the disruption of these pathways can lead to neurodegenerative disease.Keywords
This publication has 330 references indexed in Scilit:
- C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA fociActa Neuropathologica, 2013
- RNA-binding proteins in microsatellite expansion disorders: Mediators of RNA toxicityBrain Research, 2012
- Upf1 ATPase-Dependent mRNP Disassembly Is Required for Completion of Nonsense- Mediated mRNA DecayCell, 2010
- Identification of active loci of a human endogenous retrovirus in neurons of patients with amyotrophic lateral sclerosisAnnals of Neurology, 2010
- Upf1 Senses 3′UTR Length to Potentiate mRNA DecayCell, 2010
- mRNA Export from Mammalian Cell Nuclei Is Dependent on GANPCurrent Biology, 2010
- UIF, a New mRNA Export Adaptor that Works Together with REF/ALY, Requires FACT for Recruitment to mRNACurrent Biology, 2009
- Age-Dependent Deterioration of Nuclear Pore Complexes Causes a Loss of Nuclear Integrity in Postmitotic CellsCell, 2009
- Molecular characterization of novel progranulin (GRN) mutations in frontotemporal dementiaHuman Mutation, 2008
- The EJC Factor eIF4AIII Modulates Synaptic Strength and Neuronal Protein ExpressionCell, 2007