Featured Research

from universities, journals, and other organizations

Production of toxic protein causes common neurodegenerative disorder

Date:
April 18, 2013
Source:
Cell Press
Summary:
Researchers have recently discovered that an expansion of DNA in patients with the common neurodegenerative disorder Fragile X-associated tremor syndrome causes the production of an abnormal protein that is toxic to neurons. The findings suggest an unexpected process by which DNA expansions might lead to neurodegenerative diseases -- including Huntington's disease and ALS. This discovery reveals a common feature among these diseases that could be targeted to treat affected individuals.

Researchers have recently discovered that an expansion of DNA in patients with the common neurodegenerative disorder Fragile X-associated Tremor syndrome causes the production of an abnormal protein that is toxic to neurons. The findings, which are reported online April 18 in the Cell Press journal Neuron, suggest an unexpected process by which DNA expansions might lead to neurodegenerative diseases -- including Huntington's disease and ALS. This discovery reveals a common feature among these diseases that could be targeted to treat affected individuals.

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.


Story Source:

The above story is based on materials provided by Cell Press. Note: Materials may be edited for content and length.


Journal Reference:

  1. PeterK. Todd, SeokYoon Oh, Amy Krans, Fang He, Chantal Sellier, Michelle Frazer, AbigailJ. Renoux, Kai-chun Chen, K.Matthew Scaglione, Venkatesha Basrur, Kojo Elenitoba-Johnson, JeanP. Vonsattel, ElanD. Louis, MichaelA. Sutton, J.Paul Taylor, RyanE. Mills, Nicholas Charlet-Berguerand, HenryL. Paulson. CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome. Neuron, 2013; DOI: 10.1016/j.neuron.2013.03.026

Cite This Page:

Cell Press. "Production of toxic protein causes common neurodegenerative disorder." ScienceDaily. ScienceDaily, 18 April 2013. <www.sciencedaily.com/releases/2013/04/130418124856.htm>.
Cell Press. (2013, April 18). Production of toxic protein causes common neurodegenerative disorder. ScienceDaily. Retrieved October 23, 2014 from www.sciencedaily.com/releases/2013/04/130418124856.htm
Cell Press. "Production of toxic protein causes common neurodegenerative disorder." ScienceDaily. www.sciencedaily.com/releases/2013/04/130418124856.htm (accessed October 23, 2014).

Share This



More Mind & Brain News

Thursday, October 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Working Mother Getaway: Beaches Turks & Caicos

Working Mother Getaway: Beaches Turks & Caicos

Working Mother (Oct. 22, 2014) Feast your eyes on this gorgeous family-friendly resort. Video provided by Working Mother
Powered by NewsLook.com
What Your Favorite Color Says About You

What Your Favorite Color Says About You

Buzz60 (Oct. 22, 2014) We all have one color we love to wear, and believe it or not, your color preference may reveal some of your character traits. In celebration of National Color Day, Krystin Goodwin (@kyrstingoodwin) highlights what your favorite colors may say about you. Video provided by Buzz60
Powered by NewsLook.com
First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

Newsy (Oct. 21, 2014) A medical team has for the first time given a man the ability to walk again after transplanting cells from his brain onto his severed spinal cord. Video provided by Newsy
Powered by NewsLook.com
Portable Breathalyzer Gets You Home Safely

Portable Breathalyzer Gets You Home Safely

Buzz60 (Oct. 21, 2014) Breeze, a portable breathalyzer, gets you home safely by instantly showing your blood alcohol content, and with one tap, lets you call an Uber, a cab or a friend from your contact list to pick you up. Sean Dowling (@SeanDowlingTV) has the details. Video provided by Buzz60
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

More Coverage


Toxic Protein Made in Unusual Way May Explain Brain Disorder

Apr. 18, 2013 A bizarre twist on the usual way proteins are made may explain mysterious symptoms in the grandparents of some children with mental disabilities. The discovery may lead to better treatments for older ... read more

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins