ALS and the Ice Bucket Challenge: A Primer

The ALS Ice Bucket Challenge has raised over US$100 million in donations so far. But what is the Ice Bucket Challenge, and what is ALS?

AsianScientist (Sep. 5, 2014) – The social media milieu has been rife with chatter about the Ice Bucket Challenge, wherein participants douse themselves with ice water in the name of charity — namely, raising funds for and increasing awareness of amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease.

Have you decided to take up the Challenge? Here’s what you need to do:

  1. Make a donation to the ALS cause; beneficiaries include the ALS Association.
  2. Gather your supplies: a bucket, trays of ice cubes, water, and a video recording device.
  3. Announce yourself on camera! State your name, and your acceptance of the challenge.
  4. Tip the water over head.
  5. Challenge three or more people to join in the fun.
  6. Upload your video to social media. Don’t forget to use the hashtags #ALSicebucketchallenge and #icebucketchallenge
Credit: Statista.
Credit: Statista.

Popular culture aside, what should we know about ALS? ALS is a neurodegenerative disease characterized by progressive destruction of motor nerve cells in the brain and spinal cord. These nerve cells, known as motor neurons, are essential to the functioning of the body’s muscles.

As motor neurons lose their function in ALS, they are no longer able to transmit signals to and from the brain and spinal cord. As the muscle tissue weakens and atrophies, movement is lost. Initial symptoms include difficulty in swallowing, breathing and talking. These can eventually lead into total paralysis.

Most cases of ALS do not yet have a known cause; however, about ten percent are thought to result from genetic abnormalities. For example, mutations in the gene expressing the SOD1 enzyme were found to be associated with some cases of familial ALS. Although it is still not clear how mutations in the SOD1 gene lead to motor neuron degeneration, there is increasing evidence that mutant SOD1 protein can be toxic to the body.

No cure has yet been found for the disease. However, treatment with a drug known as riluzole is thought to reduce damage to motor neurons by inhibiting glutamate expression. Riluzole has been demonstrated to prolong life in certain patients, albeit only by a few months, and successfully delay severe symptoms in other patients. However, the drug is incapable of reversing damage that has already been done to motor neurons.

Ongoing research in ALS include animal studies exploring the effects of silencing the SOD1 gene mutation. An experimental drug, derived from stem cells, is also presently under testing; scientists hope that the stem cells will both protect and repair the motor neurons of ALS patients.

Researchers are also looking to re-purpose existing drugs. These include retigabine, which is used against seizures, and mexiletine, which treats cardiac defects. Both drugs work similarly to riluzole, but researchers hope these will be more effective than that drug in slowing the progression of ALS.

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Copyright: Asian Scientist Magazine; Photo: quintanomedia/Flickr/CC.
Disclaimer: This article does not necessarily reflect the views of AsianScientist or its staff.

Yamini graduated with a bachelors degree in biomedical sciences from the University of Manchester, UK. She has a passion for science and how it is perceived by the wider community.

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