Featured Research

from universities, journals, and other organizations

Findings point toward one of first therapies for Lou Gehrig's disease

Date:
June 12, 2014
Source:
Oregon State University
Summary:
Researchers have determined that a copper compound known for decades may form the basis for a therapy for amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease. In humans, prior to this, no therapy for ALS has ever been discovered that could extend lifespan more than a few additional months.

Researchers have determined that a copper compound known for decades may form the basis for a therapy for amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease.

In a new study just published in the Journal of Neuroscience, scientists from Australia, the United States (Oregon), and the United Kingdom showed in laboratory animal tests that oral intake of this compound significantly extended the lifespan and improved the locomotor function of transgenic mice that are genetically engineered to develop this debilitating and terminal disease.

In humans, no therapy for ALS has ever been discovered that could extend lifespan more than a few additional months. Researchers in the Linus Pauling Institute at Oregon State University say this approach has the potential to change that, and may have value against Parkinson's disease as well.

"We believe that with further improvements, and following necessary human clinical trials for safety and efficacy, this could provide a valuable new therapy for ALS and perhaps Parkinson's disease," said Joseph Beckman, a distinguished professor of biochemistry and biophysics in the OSU College of Science.

"I'm very optimistic," said Beckman, who received the 2012 Discovery Award from the OHSU Medical Research Foundation as the leading medical researcher in Oregon.

ALS was first identified as a progressive and fatal neurodegenerative disease in the late 1800s and gained international recognition in 1939 when it was diagnosed in American baseball legend Lou Gehrig. It's known to be caused by motor neurons in the spinal cord deteriorating and dying, and has been traced to mutations in copper, zinc superoxide dismutase, or SOD1. Ordinarily, superoxide dismutase is an antioxidant whose proper function is essential to life.

When SOD1 is lacking its metal co-factors, it "unfolds" and becomes toxic, leading to the death of motor neurons. The metals copper and zinc are important in stabilizing this protein, and can help it remain folded more than 200 years.

"The damage from ALS is happening primarily in the spinal cord and that's also one of the most difficult places in the body to absorb copper," Beckman said. "Copper itself is necessary but can be toxic, so its levels are tightly controlled in the body. The therapy we're working toward delivers copper selectively into the cells in the spinal cord that actually need it. Otherwise, the compound keeps copper inert."

"This is a safe way to deliver a micronutrient like copper exactly where it is needed," Beckman said.

By restoring a proper balance of copper into the brain and spinal cord, scientists believe they are stabilizing the superoxide dismutase in its mature form, while improving the function of mitochondria. This has already extended the lifespan of affected mice by 26 percent, and with continued research the scientists hope to achieve even more extension.

The compound that does this is called copper (ATSM), has been studied for use in some cancer treatments, and is relatively inexpensive to produce.

"In this case, the result was just the opposite of what one might have expected," said Blaine Roberts, lead author on the study and a research fellow at the University of Melbourne, who received his doctorate at OSU working with Beckman.

"The treatment increased the amount of mutant SOD, and by accepted dogma this means the animals should get worse," he said. "But in this case, they got a lot better. This is because we're making a targeted delivery of copper just to the cells that need it.

"This study opens up a previously neglected avenue for new disease therapies, for ALS and other neurodegenerative disease," Roberts said.

Other collaborators on this research include OSU, the University of Melbourne, University of Technology/Sydney, Deakin University, the Australian National University, and the University of Leeds in the United Kingdom.


Story Source:

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


Journal Reference:

  1. B. R. Roberts, N. K. H. Lim, E. J. McAllum, P. S. Donnelly, D. J. Hare, P. A. Doble, B. J. Turner, K. A. Price, S. Chun Lim, B. M. Paterson, J. L. Hickey, T. W. Rhoads, J. R. Williams, K. M. Kanninen, L. W. Hung, J. R. Liddell, A. Grubman, J.-F. Monty, R. M. Llanos, D. R. Kramer, J. F. B. Mercer, A. I. Bush, C. L. Masters, J. A. Duce, Q.-X. Li, J. S. Beckman, K. J. Barnham, A. R. White, P. J. Crouch. Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis. Journal of Neuroscience, 2014; 34 (23): 8021 DOI: 10.1523/JNEUROSCI.4196-13.2014

Cite This Page:

Oregon State University. "Findings point toward one of first therapies for Lou Gehrig's disease." ScienceDaily. ScienceDaily, 12 June 2014. <www.sciencedaily.com/releases/2014/06/140612142352.htm>.
Oregon State University. (2014, June 12). Findings point toward one of first therapies for Lou Gehrig's disease. ScienceDaily. Retrieved September 23, 2014 from www.sciencedaily.com/releases/2014/06/140612142352.htm
Oregon State University. "Findings point toward one of first therapies for Lou Gehrig's disease." ScienceDaily. www.sciencedaily.com/releases/2014/06/140612142352.htm (accessed September 23, 2014).

Share This



More Health & Medicine News

Tuesday, September 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Liberia Pleads for Help to Fight Ebola

Liberia Pleads for Help to Fight Ebola

AP (Sep. 22, 2014) Liberia's finance minister is urging the international community to quickly follow through on pledges of cash to battle Ebola. Bodies are piling up in the capital Monrovia as the nation awaits more help. (Sept. 22) Video provided by AP
Powered by NewsLook.com
Ebola Doctor Says Border Controls Critical

Ebola Doctor Says Border Controls Critical

AP (Sep. 22, 2014) A Florida doctor who helped fight the expanding Ebola outbreak in West Africa says the disease can be stopped, but only if nations quickly step up their response and make border control a priority. (Sept. 22) Video provided by AP
Powered by NewsLook.com
Global Ebola Aid Increasing But Critics Say It's Late

Global Ebola Aid Increasing But Critics Say It's Late

Newsy (Sep. 21, 2014) More than 100 tons of medical supplies were sent to West Africa on Saturday, but aid workers say the global response is still sluggish. Video provided by Newsy
Powered by NewsLook.com
Sierra Leone in Lockdown to Control Ebola

Sierra Leone in Lockdown to Control Ebola

AP (Sep. 21, 2014) Sierra Leone residents remained in lockdown on Saturday as part of a massive effort to confine millions of people to their homes in a bid to stem the biggest Ebola outbreak in history. (Sept. 20) Video provided by AP
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