Common Neurodegenerative Disorder Caused By Production Of Toxic Protein
The length of this particular DNA region is short and is not read, or translated, into a protein in normal individuals. "What we found surprised us - in cell culture models and in fly models of the human disease, the DNA expansion was in fact being translated into an aberrant protein that we call FMR1polyG," says first author Dr. Peter Todd, of the University of Michigan in Ann Arbor. "This protein was not translated in the same way as typical proteins, though. Rather, the expansion allowed protein translation to begin in the absence of a typical starting signal that's normally required for this process." This abnormal protein translation event, called "RAN" translation, occurs with different disease-causing DNA expansions to produce toxic proteins.
Importantly, RAN translation of the DNA expansions in affected patients and animals led to the accumulation of toxic FMR1polyG proteins in the brain. The investigators were able to suppress toxicity to neurons in fruit flies when they blocked production of the FMRpolyG protein. Conversely, toxicity to neurons was enhanced when they increased the protein's production.
"We were able to demonstrate that the ability to generate FMRpolyG was critical to elicit toxicity, suggesting that RAN translation is important in Fragile X-associated tremor ataxia syndrome and potentially other neurodegenerative disorders," says Dr. Todd.
Neuron, Todd et al.: "CGG Repeat Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome."
Source: EurekAlert!, the online, global news service operated by AAAS, the science society
Please use one of the following formats to cite this article in your essay, paper or report:
Press, Cell. "Common Neurodegenerative Disorder Caused By Production Of Toxic Protein." Medical News Today. MediLexicon, Intl., 22 Apr. 2013. Web.
26 Apr. 2017. <http://www.medicalnewstoday.com/releases/259352.php>
Press, C. (2013, April 22). "Common Neurodegenerative Disorder Caused By Production Of Toxic Protein." Medical News Today. Retrieved from
Please note: If no author information is provided, the source is cited instead.
Contact our news editors
For any corrections of factual information, or to contact our editorial team, please see our contact page.
Copyright Medical News Today: Excluding email/sharing services explicitly offered on this website, material published on Medical News Today may not be reproduced, or distributed without the prior written permission of Medilexicon International Ltd. Please contact us for further details.