Featured Research

from universities, journals, and other organizations

Researchers discover how ALS spreads

Date:
February 18, 2014
Source:
University of British Columbia
Summary:
The fatal neurodegenerative disease ALS, also known as Lou Gehrig's disease, has been under investigation by researchers, and in particular, how it spreads. New findings suggest that transmission can be blocked, and that misfolded non-mutant SOD1 can be transmitted from region to region in the nervous system, offering a molecular explanation for the progressive nature of the spread. ALS is a disease that affects nerve cells in the brain and the spinal cord. There are approximately 140,000 new cases diagnosed worldwide each year.

A study led by University of British Columbia and Vancouver Coastal Health Research Institute researchers has revealed how the fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is transmitted from cell to cell, and suggests the spread of the disease could be blocked.

Related Articles


"This work identifies an important piece of the puzzle in determining how the disease is transmitted throughout the nervous system," says lead investigator Dr. Neil Cashman, UBC's Canada Research Chair in Neurodegeneration and Protein Misfolding. "By understanding how this occurs, we can devise the best ways to stop the progressive neurological damage seen in ALS."

The research shows that misfolded non-mutant SOD1 can be transmitted from region to region in the nervous system, offering a molecular explanation for the progressive spread of ALS.

Published today in the Proceedings of the National Academy of Sciences, the study also shows the spread can be blocked using antibodies. Antibodies were specifically raised to bind to regions of SOD1 exposed when it is misfolded, and block its spread. If non-mutant SOD1 misfolding is the cause of ALS, as the study suggests, then the antibodies could arrest ALS progression, the researchers say.

This work builds on previous research in Cashman's lab. ALS is associated with the mutant SOD1 protein (superoxide dismutase 1) and earlier investigations found that the disease-associated mutant SOD1 can induce a change in the shape of other proteins at the molecular level by misfolding inside living cells. The affected proteins then accumulate in ways similar to the process underlying prion diseases -- rare, fatal, degenerative brain disorders seen in both humans and animals.

ALS is a disease that affects nerve cells in the brain and the spinal cord. Motor neurons progressively degenerate and die so that the brain can no longer initiate and control muscle movement. Patients in the later stages of the disease may become totally paralyzed. There are approximately 140,000 new cases diagnosed worldwide each year.


Story Source:

The above story is based on materials provided by University of British Columbia. Note: Materials may be edited for content and length.


Journal Reference:

  1. L. I. Grad, J. J. Yerbury, B. J. Turner, W. C. Guest, E. Pokrishevsky, M. A. O'Neill, A. Yanai, J. M. Silverman, R. Zeineddine, L. Corcoran, J. R. Kumita, L. M. Luheshi, M. Yousefi, B. M. Coleman, A. F. Hill, S. S. Plotkin, I. R. Mackenzie, N. R. Cashman. Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms. Proceedings of the National Academy of Sciences, 2014; DOI: 10.1073/pnas.1312245111

Cite This Page:

University of British Columbia. "Researchers discover how ALS spreads." ScienceDaily. ScienceDaily, 18 February 2014. <www.sciencedaily.com/releases/2014/02/140218114305.htm>.
University of British Columbia. (2014, February 18). Researchers discover how ALS spreads. ScienceDaily. Retrieved November 28, 2014 from www.sciencedaily.com/releases/2014/02/140218114305.htm
University of British Columbia. "Researchers discover how ALS spreads." ScienceDaily. www.sciencedaily.com/releases/2014/02/140218114305.htm (accessed November 28, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Friday, November 28, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola Leaves Orphans Alone in Sierra Leone

Ebola Leaves Orphans Alone in Sierra Leone

AFP (Nov. 27, 2014) — The Ebola epidemic sweeping Sierra Leone is having a profound effect on the country's children, many of whom have been left without any family members to support them. Duration: 01:02 Video provided by AFP
Powered by NewsLook.com
Experimental Ebola Vaccine Shows Promise In Human Trial

Experimental Ebola Vaccine Shows Promise In Human Trial

Newsy (Nov. 27, 2014) — A recent test of a prototype Ebola vaccine generated an immune response to the disease in subjects. Video provided by Newsy
Powered by NewsLook.com
Pet Dogs to Be Used in Anti-Ageing Trial

Pet Dogs to Be Used in Anti-Ageing Trial

Reuters - Innovations Video Online (Nov. 26, 2014) — Researchers in the United States are preparing to discover whether a drug commonly used in human organ transplants can extend the lifespan and health quality of pet dogs. Video provided by Reuters
Powered by NewsLook.com
Today's Prostheses Are More Capable Than Ever

Today's Prostheses Are More Capable Than Ever

Newsy (Nov. 26, 2014) — Advances in prosthetics are making replacement body parts stronger and more lifelike than they’ve ever been. Video provided by Newsy
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