Featured Research

from universities, journals, and other organizations

Misfolded Protein Clumps Found To Be Common To Dementia, Lou Gehrig's Disease

Date:
October 6, 2006
Source:
NIH/National Institute on Aging
Summary:
Scientists funded by the National Institute on Aging have identified a protein common to two neurodegenerative diseases -- frontotemporal dementia and Lou Gehrig's disease. This discovery suggests that these diseases might share a common pathological process.

Misfolded disease proteins in neurons of the hippocampus.
Credit: Virginia M-Y. Lee, Ph.D. University of Pennsylvania School of Medicine; Science

Scientists have identified a misfolded, or incorrectly formed, protein common to two devastating neurological diseases, frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease), according to a report in the Oct. 6, 2006, issue of Science. The findings suggest that certain forms of FTD, ALS and possibly other neurological diseases might share a common pathological process.

Related Articles


Virginia Lee, Ph.D., and John Trojanowski, M.D., Ph.D., of the University of Pennsylvania, led an international team of scientists in this discovery. The work was funded by the National Institute on Aging (NIA), part of the National Institutes of Health (NIH), and was done at the NIA-funded Alzheimer's Disease Center at the University of Pennsylvania School of Medicine Institute on Aging.

"This exciting basic science discovery provides the first molecular link between a dementia--FTD--and a motor neuron disease--ALS. It will advance understanding of the pathological processes of FTD and ALS, and possibly of other neurological disorders," says NIA director Richard J. Hodes, M.D. Improved understanding of underlying disease processes is critically important in pointing researchers toward the development of therapies for FTD, ALS and other neurodegenerative diseases, Hodes and the study authors note.

FTD affects the frontal and temporal lobes of the brain. People with FTD may exhibit uninhibited and socially inappropriate behavior, changes in personality and, in late stages, loss of memory, motor skills and speech. After Alzheimer's disease, it is the most common cause of dementia in people under age 65.

ALS is a progressive disease of brain and spinal cord motor neurons that control movement. Over time, walking, eating, speaking and breathing become more difficult in this fatal disease. Some people with ALS also have FTD, and some with FTD also develop ALS, suggesting that common mechanisms might underlie these two diseases.

In certain neurodegenerative diseases, including ALS and some forms of FTD, scientists have identified clumps of protein--or inclusion bodies--that accumulate in brain cells and neurons. However, understanding why they form and what they contain has been elusive. Lee and Trojanowski have long sought to solve that mystery.

Following years of research, they have now identified TDP-43 as a constituent part of the clumps that form in ALS and in the most common form of FTD. Although its precise role is not well understood, TDP-43 is involved in the complex process of transcribing and regulating genetic information in the nucleus of the cell.

"There is much more to learn about how this nuclear protein is clumped in the cytoplasm of cells and about the mechanism by which it is implicated in two distinctly different diseases," says Stephen Snyder, Ph.D., program director, etiology of Alzheimer's disease, NIA Neuroscience and Neuropsychology of Aging Program. "It is possible that the TDP-43 protein will be a key to a more complete understanding of both FTD and ALS."


Story Source:

The above story is based on materials provided by NIH/National Institute on Aging. Note: Materials may be edited for content and length.


Cite This Page:

NIH/National Institute on Aging. "Misfolded Protein Clumps Found To Be Common To Dementia, Lou Gehrig's Disease." ScienceDaily. ScienceDaily, 6 October 2006. <www.sciencedaily.com/releases/2006/10/061005222750.htm>.
NIH/National Institute on Aging. (2006, October 6). Misfolded Protein Clumps Found To Be Common To Dementia, Lou Gehrig's Disease. ScienceDaily. Retrieved January 31, 2015 from www.sciencedaily.com/releases/2006/10/061005222750.htm
NIH/National Institute on Aging. "Misfolded Protein Clumps Found To Be Common To Dementia, Lou Gehrig's Disease." ScienceDaily. www.sciencedaily.com/releases/2006/10/061005222750.htm (accessed January 31, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Saturday, January 31, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

CDC: Get Vaccinated for Measles

CDC: Get Vaccinated for Measles

Reuters - US Online Video (Jan. 30, 2015) The CDC is urging people to get vaccinated for measles amid an outbreak that began at Disneyland and has now infected more than 90 people. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Obama To Outline New Plan For Personalized Medicine

Obama To Outline New Plan For Personalized Medicine

Newsy (Jan. 30, 2015) President Obama is expected to speak with drugmakers Friday about his Precision Medicine Initiative first introduced last week. Video provided by Newsy
Powered by NewsLook.com
NFL Concussions Down; Still on Parents' Minds

NFL Concussions Down; Still on Parents' Minds

AP (Jan. 30, 2015) The NFL announced this week that the number of game concussions dropped by a quarter over last season. Still, the dangers of the sport still weigh on players, and parents&apos; minds. (Jan. 30) Video provided by AP
Powered by NewsLook.com
U.S. Wants to Analyze DNA from 1 Million People

U.S. Wants to Analyze DNA from 1 Million People

Reuters - US Online Video (Jan. 30, 2015) The U.S. has proposed analyzing genetic information from more than 1 million American volunteers to learn how genetic variants affect health and disease. Rough Cut (no reporter narration). Video provided by Reuters
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:

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