Preserving nerve cells in motor neuron disease

Sep 20, 2010

A team of researchers, led by Scott Oakes, at the University of California, San Francisco, has identified a way to prevent symptom onset, weight loss, and paralysis and extend survival in a mouse model of amyotrophic lateral sclerosis (ALS; also known as Lou Gehrig's disease), providing a new avenue of research for the development of therapeutics for ALS and other motor neuron diseases.

ALS and other motor neuron diseases are neurological disorders that selectively affect that control voluntary muscle activities such as speaking, walking, breathing, swallowing, and general movement of the body. A key feature of these diseases is that the affected nerve cells (which are known as motor neurons) die by a process known as apoptosis.

Determining whether this death contributes to disease or occurs after the nerves have stopped functioning is important to establishing whether blocking apoptosis would have therapeutic benefit. In the study, genetically eliminating activation of the mitochondrial apoptotic pathway in a of ALS was shown to preserve motor neuron viability and function, thereby preventing symptom onset, weight loss, and and extending survival.

The authors therefore suggest that inhibiting activation of the mitochondrial apoptotic pathway might provide a way to preserve motor neurons in individuals with ALS and other motor neuron diseases.

Explore further: Built for speed: paranodal junction assembly in high performance nerves

More information: View this article at: www.jci.org/articles/view/4298… b51352dad375af860586

Provided by Journal of Clinical Investigation

5 /5 (2 votes)
add to favorites email to friend print save as pdf

Related Stories

Genetics of ALS progression

Jun 01, 2008

An upcoming paper in the June 1 issue of G&D, from Drs. Hidenori Ichijo and Hideki Nishitoh (University of Tokyo) and colleagues, lends new and valuable insight into the genetics of ALS.

Researchers discover genetic link between both types of ALS

May 05, 2010

Researchers from Northwestern University Feinberg School of Medicine have discovered a link between sporadic and familial forms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease also known as Lou Gehrig's ...

Research suggests new direction for ALS treatment

Nov 28, 2007

A research team from Wake Forest University School of Medicine is the first to show that injections of a protein normally found in human cells can increase lifespan and delay the onset of symptoms in mice with ALS (amyotrophic ...

Recommended for you

Damage to brain networks affects stroke recovery

Nov 21, 2014

(Medical Xpress)—Initial results of an innovative study may significantly change how some patients are evaluated after a stroke, according to researchers at Washington University School of Medicine in St. ...

Dopamine leaves its mark in brain scans

Nov 21, 2014

Researchers use functional magnetic resonance imaging (fMRI) to identify which areas of the brain are active during specific tasks. The method reveals areas of the brain, in which energy use and hence oxygen ...

User comments : 0

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.