RNA Nucleocytoplasmic Transport Defects in Neurodegenerative Diseases
Document Type
Article
Abstract
In eukaryotic cells, transcription and translation are compartmentalized by the nuclear membrane, requiring an active transport of RNA from the nucleus into the cytoplasm. This is accomplished by a variety of transport complexes that contain either a member of the exportin family of proteins and translocation fueled by GTP hydrolysis or in the case of mRNA by complexes containing the export protein NXF1. Recent evidence indicates that RNA transport is altered in a number of different neurodegenerative diseases including Huntington's disease, Alzheimer's disease, frontotemporal dementia, and amyotrophic lateral sclerosis. Alterations in RNA transport predominately fall into three categories: Alterations in the nuclear membrane and mislocalization and aggregation of the nucleoporins that make up the nuclear pore; alterations in the Ran gradient and the proteins that control it which impacts exportin based nuclear export; and alterations of proteins that are required for the export of mRNA leading nuclear accumulation of mRNA.
Keywords
Alzheimer’s disease, Amyotrophic lateral sclerosis, Exportin, Frontotemporal dementia, Huntington’s disease, Nuclear pore complex, RNA, TREX
Medical Subject Headings
Humans; Neurodegenerative Diseases (metabolism); Nuclear Envelope (metabolism); Nuclear Pore (metabolism); Nuclear Pore Complex Proteins (metabolism); RNA Transport (physiology)
Publication Date
6-20-2018
Publication Title
Advances in neurobiology
ISSN
2190-5215
Volume
20
First Page
85
Last Page
101
PubMed ID
29916017
Digital Object Identifier (DOI)
10.1007/978-3-319-89689-2_4
Recommended Citation
Boehringer, Ashley and Bowser, Robert, "RNA Nucleocytoplasmic Transport Defects in Neurodegenerative Diseases" (2018). Translational Neuroscience. 1057.
https://scholar.barrowneuro.org/neurobiology/1057