Navigation Links
Finding Raises Hope for Treating ALS
Date:6/15/2009

Time between onset and symptoms could be targeted for therapy, study suggests,,

MONDAY, JUNE 15 (HealthDay News) -- Researchers now believe that ALS, or amyotrophic lateral sclerosis, starts well before its debilitating symptoms appear, a finding that they say could eventually lead to an effective treatment.

Protein clumps in cells that show up only after damage has occurred, rather than at the onset of the disease, appear responsible for the paralyzing course of ALS (amyotrophic lateral sclerosis), according to University of Florida researchers.

The research team found that the formation of these clumps of defective proteins and cell matter, called "protein aggregates," actually signals that ALS is progressing at a rapid pace.

In ALS, nerve cells extending from the brain to the spinal cord and then to the muscles die off. Its cause is unknown, though an inherited defective gene is suspected in up to 20 percent of cases. Scientists thought it originated with malformed -- or "misfolded" -- proteins that malfunction and lead to the protein aggregates in the brain.

The ALS Association says that about 30,000 Americans have the disease, which is often referred to as Lou Gehrig's disease, after the renowned ballplayer who died of ALS.

The Florida researchers examined the gene that produces superoxide dismutase 1, or SOD1, an enzyme that fights off free radicals -- molecules that damage the body's cells. People with the inherited version of ALS have been found to have one of the 146 known mutations in the SOD1 gene.

In the online edition of Human Molecular Genetics, the researchers wrote that the SOD1 mutations most likely to cause the protein clumps were also linked to the disease's faster progression.

Another study, recently published online in the Proceedings of the National Academy of Sciences, found that mice genetically altered to have ALS experience cell damage well before protein aggregates start to appear, a time that coincides with telltale symptoms of the disease.

"As the disease enters the symptomatic stage in mice, the buildup of protein is rapid and dramatic," David Borchelt, director of the SantaFe HealthCare Alzheimer's Disease Research Center at the university's McKnight Brain Institute, where the studies took place, said in a university news release. "However, the formation of these aggregates is not the whole story. It is well-established that significant damage to the nervous system occurs well before the symptoms appear. The uncontrolled misfolding of SOD1 seems to be confined to the late stage of disease, which is when symptoms first appear, giving hope that treatments targeting this process could be beneficial."

The finding suggests that there may be significant time to treat ALS before the clumping and symptoms appear, signaling the quickening pace of progression. But this would require development of an accurate way to diagnose ALS before symptoms emerge.

"Blocking aggregation of these proteins could be a therapeutic target for individuals with this genetic mutation," Borchelt said. "Right now, there is little that can be done to help these patients."

More information

The ALS Association has more about ALS.



-- Kevin McKeever



SOURCE: University of Florida, news release, June 8, 2009


'/>"/>
Copyright©2009 ScoutNews,LLC.
All rights reserved  

Related medicine news :

1. Perrier Refutes Consumer Council Findings
2. Finding May Lead to Vaccine for Travelers Diarrhea
3. Finding Fitness on the Dance Floor
4. Findings in Epilepsy Gene in Animals May Guide Treatment Directions for Infants
5. Findings in epilepsy gene in animals may guide treatment directions for infants
6. Pitt melanoma researchers present novel findings at ASCO
7. Finding Hidden Heart Attacks: Saint Francis Hospital is First in Illinois to Detect Hidden Heart Attacks With New Technology
8. Finding a New Doctor Becoming More Difficult for Medicare Recipients Says MedicareSupplementPlans.com
9. Statement by American Legacy Foundation(R): Findings of Decades of Massive Fraud by Tobacco Industry Upheld in U.S. Court of Appeals
10. HIMSS Analytics and NetMotion Wireless Release Study Findings on Key Challenges for Wireless Healthcare Deployments
11. UCSF creates fast, affordable tool for finding gene on-off switches
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Finding Raises Hope for Treating ALS
(Date:4/28/2017)... ... April 28, 2017 , ... The Texas Cord Blood Bank (TCBB), ... labor and delivery team at Women’s Hospital at Renaissance in Edinburg for their outstanding ... the hospital and decide to donate. , “Women’s Hospital at Renaissance has been ...
(Date:4/28/2017)... ... April 28, 2017 , ... Early detection and elimination ... safety and minimize the cost of development. In this webinar, sponsored by Molecular ... lines and for cardiac toxicity using induced pluripotent stem cells (iPSCs). , In ...
(Date:4/28/2017)... ... ... Bill Howe started his sewer and drain company in 1980 focusing heavily ... team, the Bill Howe brand was born and they began cultivating their mission to ... the San Diego community in which they worked, lived and were raising their daughters. ...
(Date:4/28/2017)... ... April 28, 2017 , ... Researchers at ... of ActiGraph’s CentrePoint Data Hub in a sample of participants enrolled ... wearable activity and sleep monitoring solutions for the global scientific community. The company’s ...
(Date:4/28/2017)... ... 2017 , ... Intellitec Solutions announced the publication of a ... Dynamics GP solution that integrates to their PointClickCare EHR software package. With the ... Brooke Grove now has the capability to achieve its goal for a comprehensive ...
Breaking Medicine News(10 mins):
(Date:4/20/2017)...  Zimmer Biomet Holdings, Inc. (NYSE and SIX: ZBH), ... it will be participating in the Deutsche Bank Securities ... in Boston, Massachusetts on Wednesday, ... a.m. Eastern Time. A live webcast of ... Relations website at http://investor.zimmerbiomet.com .  The webcast will ...
(Date:4/20/2017)... April 20, 2017 Research and Markets has ... Innovation Driven by Rapidly Expanding Injectables Market and Increasing Usage of ... ... delivery technologies will rise from USD 20 Billion in 2015 to ... Drug Delivery Technologies - Innovation Driven by Rapidly Expanding Injectables Market ...
(Date:4/20/2017)... 20, 2017  AbbVie (NYSE: ABBV), a global ... of chronic hepatitis C virus (HCV) infected patients ... and compensated cirrhosis (Child-Pugh A) achieved sustained virologic ... with its investigational, pan-genotypic regimen of glecaprevir/pibrentasvir (G/P). ... following 12 weeks of G/P treatment without ribavirin. ...
Breaking Medicine Technology: