학술논문

Regulation of RNA granules by FMRP and implications for neurological diseases.
Document Type
Article
Source
Traffic. Jul2020, Vol. 21 Issue 7, p454-462. 9p.
Subject
*NEUROLOGICAL disorders
*RNA
*AMYOTROPHIC lateral sclerosis
*RNA-binding proteins
*INTELLECTUAL disabilities
Language
ISSN
1398-9219
Abstract
RNA granule formation, which can be regulated by RNA‐binding proteins (RBPs) such as fragile X mental retardation protein (FMRP), acts as a mechanism to control both the repression and subcellular localization of translation. Dysregulated assembly of RNA granules has been implicated in multiple neurological disorders, such as amyotrophic lateral sclerosis. Thus, it is crucial to understand the cellular pathways impinging upon granule assembly or disassembly. The goal of this review is to summarize recent advances in our understanding of the role of the RBP, FMRP, in translational repression underlying RNA granule dynamics, mRNA transport and localized. We summarize the known mechanisms of translational regulation by FMRP, the role of FMRP in RNA transport granules, fragile X granules and stress granules. Focusing on the emerging link between FMRP and stress granules, we propose a model for how hyperassembly and hypoassembly of RNA granules may contribute to neurological diseases. [ABSTRACT FROM AUTHOR]