Scientists get to work on ALS with ice bucket challenge donations


George Bush before and after taking the ice bucket challenge. The campaign raised $115 million worldwide
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The ice bucket challenge craze to raise awareness of amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease, has died down, but while it raged the viral video campaign raised $115 million from more than three million donors.

By any measures it was a success. As Scientific American points out, from July 29 to August 29, donors contributed $100.9 million, compared with $2.8 million during the same period the previous year.

Now the focus has turned to what research the money raised will be used for.

In early October, the ALS Association began spending that money. It approved $21.7 million of funding for six programs and initiatives by groups that include the academic-industry partnership ALS Accelerated Therapeutics, the New York Genome Center, three California labs that form the Neuro Collaborative, and Project MinE, which will map the genomes of 15,000 people with ALS (about 10 percent of ALS patients have a family member with the disease). The grants focus on developing gene therapies for common ALS genes and exploring approaches to counter two major contributors to the disease, the inflammation of nervous tissue and misfolded proteins in brain cells that control movement.

In many ways researchers are starting at square one. Not only do scientists not know the cause of ALS, they do not even have a dominant theory from which to work. But a good starting point could be to work out why eye muscles in ALS patients continue to function after others waste away.

One clue to a better theory—and possibly new avenues of treatment—may lie in an intriguing detail about ALS: some nerve cells die from the disease, but certain others do not. ALS destroys nerve cells in the brain and spinal cord that control muscle movement. Control of arms and legs typically weakens first, followed by other muscles, such as those used for breathing and eating. In the last stages of ALS, which is typically fatal in two to five years after diagnosis, patients have lost most of their motor neurons. Yet many patients, even in late stages, can still move their eyes and sometimes control the sphincter and a few other muscles.

Scientific American suggests that it may be down to the wiring of the motor neurons.

Those vulnerable to ALS connect to specialized sensory neurons and “receive continuous excitation from other neurons that release glutamate,” an important neurotransmitter that excites motor neurons and triggers muscle movement, explains neuroscientist George Mentis of the Center for Motor Neuron Biology and Disease at Columbia University.

Eye and sphincter motor neurons, however, do not receive synaptic connections from these specialised sensory neurons and receive less glutamate.

  1. http://www.scientificamerican.com/article/why-do-eye-muscles-furnction-in-als-while-other-muscles-waste-away/
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