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.

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 July 22, 2014 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 July 22, 2014).

Share This




More Health & Medicine News

Tuesday, July 22, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Gilead's $1000-a-Pill Drug Could Cure Hep C in HIV-Positive People

Gilead's $1000-a-Pill Drug Could Cure Hep C in HIV-Positive People

TheStreet (July 21, 2014) New research shows Gilead Science's drug Sovaldi helps in curing hepatitis C in those who suffer from HIV. In a medical study, the combination of Gilead's Hep C drug with anti-viral drug Ribavirin cured 76% of HIV-positive patients suffering from the most common hepatitis C strain. Hepatitis C and related complications have been a top cause of death in HIV-positive patients. Typical medication used to treat the disease, including interferon proteins, tended to react badly with HIV drugs. However, Sovaldi's %1,000-a-pill price tag could limit the number of patients able to access the treatment. TheStreet's Keris Lahiff reports from New York. Video provided by TheStreet
Powered by NewsLook.com
$23.6 Billion Awarded To Widow In Smoking Lawsuit

$23.6 Billion Awarded To Widow In Smoking Lawsuit

Newsy (July 20, 2014) Cynthia Robinson claims R.J. Reynolds Tobacco Company hid the health and addiction risks of its products, leading to the death of her husband in 1996. Video provided by Newsy
Powered by NewsLook.com
Tooth Plaque Provides Insight Into Diets Of Ancient People

Tooth Plaque Provides Insight Into Diets Of Ancient People

Newsy (July 19, 2014) Research on plaque from ancient teeth shows that our prehistoric ancestor's had a detailed understanding of plants long before developing agriculture. Video provided by Newsy
Powered by NewsLook.com
Contaminated Water Kills 3 Babies in South African Town

Contaminated Water Kills 3 Babies in South African Town

AFP (July 18, 2014) Contaminated water in South Africa's northwestern town of Bloemhof kills three babies and hospitalises over 500 people. The incident highlights growing fears over water safety in South Africa. Duration: 02:22 Video provided by AFP
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:
from the past week

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