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
Comment
. 2013 May 7;110(19):7533-4.
doi: 10.1073/pnas.1305239110. Epub 2013 Apr 29.

Toxic RNA as a driver of disease in a common form of ALS and dementia

Harry T Orr  1
Affiliations
Comment

Toxic RNA as a driver of disease in a common form of ALS and dementia

Harry T Orr. Proc Natl Acad Sci U S A. .
No abstract available

PubMed Disclaimer

Conflict of interest statement

The author declares no conflict of interest.

Figures

Fig. 1.
Fig. 1.
Scheme depicting the three major putative mechanisms underlying expanded C9ORF72 repeat ALS/FTD. Pathway I involves a loss of function of C9ORF72 and/or other nearby genes. Both mechanisms II and III involve a toxic gain of function, with pathway II consisting of a toxic RNA and in III, toxicity being attributable to a protein or peptide.
See this image and copyright information in PMC

Comment on

References

    1. DeJesus-Hernandez M, et al. Expanded GGGGCC hexanucleotide repeat in non-coding region of C9ORF72 causes chromosome 9p-linked frontotemporal dementia and amyotrophic lateral sclerosis. Neuron. 2011;72(2):245–256. - PMC - PubMed
    1. Renton AE, et al. ITALSGEN Consortium A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011;72(2):257–268. - PMC - PubMed
    1. Gijselinck I, et al. A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: A gene identification study. Lancet Neurol. 2012;11(1):54–65. - PubMed
    1. Renoux AJ, Todd PK. Neurodegeneration the RNA way. Prog Neurobiol. 2012;97(2):173–189. - PMC - PubMed
    1. Zu T, et al. Non-ATG-initiated translation directed by microsatellite expansions. Proc Natl Acad Sci USA. 2011;108(1):260–265. - PMC - PubMed

LinkOut - more resources