they did not say how

[tdamess]

anyway to find info. on this or just another let down -

Stopping Nerve Death in ALS

Researchers have found a way to improve the lives of animals with amyotrophic lateral sclerosis (ALS).

Investigators from the University of California, San Francisco prevented symptom onset, weight loss, and paralysis in a mouse model of ALS. They were also able to extend lives.

ALS is a neurological disorder that affects cells that control voluntary muscle activities such as speaking, walking, breathing, swallowing and general movement. One characteristic of ALS is that the nerve cells die by a process known as apoptosis. Determining whether this cell death contributes to the disease or occurs after nerves stop functioning is key to establishing whether blocking apoptosis is beneficial.

In the study, genetically eliminating activation of the mitochondrial apoptotic pathway preserved nerve cell viability and function, thus helping animals with the disease.

According to the ALS Association, ALS affects more than 5,600 people in the U.S. each year. About 30,000 Americans are living with the disease at any given time.

SOURCE: Journal of Clinical Investigation, September 2010

 

[Katie C]

UCSF does really good work, so I imagine the finding is legit if very early on. You could probably search the journal of clinical investigation to find the entire article, though it's probably going to be very dry, technical reading.

 

[John1]

Re:link

I'm no biologist but as I understand the paper, the research attempts to determine whether cell apoptosis contributes to ALS progression or is a result thereof. The researchers claim that their work demonstrates that it is contributor to disease progression. However they do not offer a method to stop it. They genetically altered the ALS mice so that they would be resistant to apopstosis and although they developed ALS, progression was slightly slower and lifetimes extended. The main benefit though seems to be that disease onset is delayed by about 20 days. Once progression begins, both the "resistant" ALS mice and the normal ALS mice progress similarily except for a few in the resistant group that live about 20 days longer.

The article's abstract:

Apoptosis of motor neurons is a well-documented feature in amyotrophic lateral sclerosis (ALS) and related motor neuron diseases (MNDs). However, the role of apoptosis in the pathogenesis of these diseases remains unresolved. One possibility is that the affected motor neurons only succumb to apoptosis once they have exhausted functional capacity. If true, blocking apoptosis should confer no therapeutic benefit. To directly investigate this idea, we tested whether tissue-specific deletion in the mouse CNS of BCL2-associated X protein (BAX) and BCL2-homologous antagonist/killer (BAK), 2 proapoptotic BCL-2 family proteins that together represent an essential gateway to the mitochondrial apoptotic pathway, would protect against motor neuron degeneration. We found that neuronal deletion of Bax and Bak in a mouse model of familial ALS not only halted neuronal loss, but prevented axonal degeneration, symptom onset, weight loss, and paralysis and extended survival. These results show that motor neurons damaged in ALS activate the mitochondrial apoptotic pathway early in the disease process and that apoptotic signaling directly contributes to neuromuscular degeneration and neuronal dysfunction. Hence, inhibiting apoptosis upstream of mitochondrial permeabilization represents a possible therapeutic strategy for preserving functional motor neurons in ALS and other MNDs.

 

[tdamess]

thank you john1 you explained it so i would understand it better and you to katie for your response ... i wonder how they altered the mice