Navigation Links
Scientists ID 'Jekyll-Hyde' Protein in Lou Gehrig's Disease

Gene mutations cause superoxide dismutase to clump up in motor neurons

TUESDAY, July 29 (HealthDay News) -- A protein dysfunction could help drive amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, according to a study by researchers at Brandeis University and Harvard Medical School.

ALS is a fatal neurodegenerative disease caused by the death of motor neurons in the brain and spinal cord that control muscle movement. In this study, researchers focused on the rare, familial form of ALS (fALS) as a way of better understanding the sporadic form of ALS, which accounts for 90 percent of all cases. Clinical symptoms are identical in both forms of the disease.

About 15 years ago, scientists found that mutations in the gene that makes the protein superoxide dismutase caused familial ALS, but just how these mutations cause ALS has remained a mystery.

In this new study, researchers found that fALS is caused by two properties of superoxide dismutase that create toxic levels of the protein in motor neurons.

"We discovered that increased protein unfolding and the propensity of the proteins to aggregate (clump together) are the major factors in the familial form of ALS," Brandeis chemist Jeff Agar said in a university news release.

"The protein superoxide dismutase, normally a useful antioxidant, goes from Dr. Jekyll to Mr. Hyde when it clumps up," Agar said.

The study was published this week in the journal PLoS Biology.

The next phase of this research is to develop drugs that prevent superoxide dismutase from clumping together.

More information

The Muscular Dystrophy Association has more about ALS.

-- Robert Preidt

SOURCE: Brandeis University, news release, July 28, 2008

Copyright©2008 ScoutNews,LLC.
All rights reserved

Related medicine news :

1. New prion protein discovered by Canadian scientists may offer insight into mad cow disease
2. Scientists Probe Sepsis Deadly Secrets
3. Scientists puzzled by severe allergic reaction to cancer drug in the middle Southern US
4. Scientists Develop Natural Protection for Stored Foods
5. Scientists detect presence of marburg virus in african fruit bats
6. Scientists Spot Brains Free Will Center
7. Scientists ID Likely Culprit in Popcorn Lung
8. Scientists explain how insulin secreting cells maintain their glucose sensitivity
9. Scripps Research scientists shed new light on how antibodies fight HIV
10. Scientists, physicians present latest findings in personalized cancer treatment and prevention
11. Scientists demonstate link between genetic variant and effectiveness of smoking cessation meds
Post Your Comments:
(Date:11/25/2015)... , ... November 25, 2015 , ... ... has focused on providing comprehensive solutions involving adult stem cell therapies to patients ... officially deemed the “Regenestem” name as a Registered Trademark (RTM). , Organizations are ...
(Date:11/24/2015)... ... 2015 , ... Genesis Chiropractic Software helps practice owners automate ... between the practice owner and the patient that automatically manages all five aspects ... Click here to learn more. , According to Dr. Brian ...
(Date:11/24/2015)... ... November 24, 2015 , ... New patients who wish ... referral for dental implants at her Mississauga, ON practice. Dr. Williams has ... the placement of dental implants. , Missing teeth can lead to a variety ...
(Date:11/24/2015)... FRANCISCO, CA (PRWEB) , ... November 24, 2015 ... ... half-cup sizes, has launched their Black Friday sale a week early, offering 40% ... continues to transform the intimate apparel industry through both mobile fit technology and ...
(Date:11/24/2015)... ... November 24, 2015 , ... DMG Productions announced that they will feature ... quarter 2016 via Discovery Channel. Dates and show times TBA. , Aphria, Inc., is ... the business of producing and supplying medical marijuana pursuant to the Marijuana for Medical ...
Breaking Medicine News(10 mins):
(Date:11/25/2015)... November 25, 2015 ... addition of the  "Global Drug Device ... to their offering.  --> ... of the  "Global Drug Device Combination ... their offering.  --> Research ...
(Date:11/24/2015)... 2015 WuXi PharmaTech (Cayman) Inc. ("WuXi" or ... R&D capability and technology platform company serving the pharmaceutical, ... China and the United ... general meeting of shareholders held today, the Company,s shareholders ... approve the previously announced agreement and plan of merger ...
(Date:11/24/2015)... CARLOS, Calif. , Nov. 24, 2015 ... a leader in non-invasive genetic testing and ... announced that it will present at the ... in New York on Wednesday, December 2, 2015 at 1:00 p.m. ET.  Matthew ... the Company,s financial results, business activities and ...
Breaking Medicine Technology: