Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 May 1;10(5):509-518.
doi: 10.1242/dmm.028613.

RNA metabolism in neurodegenerative disease

Elaine Y Liu  1 , Christopher P Cali  1 , Edward B Lee  2
Affiliations
Review

RNA metabolism in neurodegenerative disease

Elaine Y Liu et al. Dis Model Mech. .

Abstract

Aging-related neurodegenerative diseases are progressive and fatal neurological diseases that are characterized by irreversible neuron loss and gliosis. With a growing population of aging individuals, there is a pressing need to better understand the basic biology underlying these diseases. Although diverse disease mechanisms have been implicated in neurodegeneration, a common theme of altered RNA processing has emerged as a unifying contributing factor to neurodegenerative disease. RNA processing includes a series of distinct processes, including RNA splicing, transport and stability, as well as the biogenesis of non-coding RNAs. Here, we highlight how some of these mechanisms are altered in neurodegenerative disease, including the mislocalization of RNA-binding proteins and their sequestration induced by microsatellite repeats, microRNA biogenesis alterations and defective tRNA biogenesis, as well as changes to long-intergenic non-coding RNAs. We also highlight potential therapeutic interventions for each of these mechanisms.

Keywords: Disease; Microsatellite repeats; RNA; RNA binding proteins; lncRNA; miRNA; tRNA.

PubMed Disclaimer

Conflict of interest statement

Competing interestsThe authors declare no competing or financial interests.

Figures

None
A high-resolution version of the poster is available for downloading at http://dmm.biologists.org/lookup/doi/10.1242/dmm.028613.supplemental.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Alami N. H., Smith R. B., Carrasco M. A., Williams L. A., Winborn C. S., Han S. S. W., Kiskinis E., Winborn B., Freibaum B. D., Kanagaraj A. et al. (2014). Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations. Neuron 81, 536-543. 10.1016/j.neuron.2013.12.018 - DOI - PMC - PubMed
    1. Anderson P. and Ivanov P. (2014). tRNA fragments in human health and disease. FEBS Lett. 588, 4297-4304. 10.1016/j.febslet.2014.09.001 - DOI - PMC - PubMed
    1. Antonellis A., Ellsworth R. E., Sambuughin N., Puls I., Abel A., Lee-Lin S.-Q., Jordanova A., Kremensky I., Christodoulou K., Middleton L. T. et al. (2003). Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V. Am. J. Hum. Genet. 72, 1293-1299. 10.1086/375039 - DOI - PMC - PubMed
    1. Aoki Y., Manzano R., Lee Y., Dafinca R., Aoki M., Douglas A. G. L., and Wood M. J. A. (2017). C9orf72 and RAB7L1 regulate vesicle trafficking in amyotrophic lateral sclerosis and frontotemporal dementia. Brain 140, 887-897. 10.1093/brain/awx024 - DOI - PubMed
    1. Ash P. E. A., Bieniek K. F., Gendron T. F., Caulfield T., Lin W.-L., DeJesus-Hernandez M., van Blitterswijk M. M., Jansen-West K., Paul J. W., Rademakers R. et al. (2013). Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 77, 639-646. 10.1016/j.neuron.2013.02.004 - DOI - PMC - PubMed

Publication types