ALS (Amyotrophic Lateral Sclerosis)
With all the social media presence on ALS (amyotrophic lateral sclerosis) with the recent ALS ice bucket challenge, we thought it was important to delve into what ALS is and what the current research offers.
Check out Dr. Serné’s ALS Ice Bucket Challenge
Current ALS Research
ALS (amyotrophic lateral sclerosis) or commonly known as Lou Gehrig’s Diesease, is the most common motor neuron disease. Both upper and lower motor neurons are affected and the disease is typically fatal. Most often symptoms are first observed in the arms and legs, where patients may notice weakness, trembling, and atrophy. The muscles responsible for speech, chewing and swallowing are also affected in about 75% of patients.
Familial ALS is associated with genetic mutations and represents 10% of those with ALS. The remaining 90% of cases are considered sporadic, meaning no genetic or familial association exists. Although several factors have been suggested, no specific triggers have been identified to date. Perhaps the most promising research surrounds the therapeutic use of L-serine. The mechanism behind L-serine’s likely therapeutic effect is actually quite interesting, and began with the observation that individuals living near bodies of water containing substantial amounts of cyanobacteria, were up to 25% more likely to develop ALS.
Dr. Paul Cox, founder of the Institute for EthnoMedicine in Jackson Hole, Wyoming, has been studying neurodegenerative disorders like ALS and dementia for some time. In 2012, he presented his findings to the world at a TEDx convention in Wyoming. Dr. Cox discovered a mechanism by which a toxin produced by cyanobacteria called BMAA (Beta-N-Methylamino-l-alaline) is incorporated into the food chain, and contributes to protein misfolding and subsequent neurodegenerative damage.
On the Island of Guam, the local people called the Chamorro, have an unusually high occurrence of ALS with components of dementia and Parkinson’s like disease. The Chamorro people eat plenty of tortillas made from local cycad seeds, containing quite a bit of cyanobacteria. The flour milled from the cycad seeds is soaked for a week to remove the majority of toxins, but still contains plenty of BMAA. Additionally, local fruit bats who feed on the cycad seeds are considered a delicacy and have been hunted to extinction in Guam, the bats themselves also contained very high levels of BMAA.
BMAA looks quite a bit like L-serine, an amino acid normally used during protein synthesis. Over time, abnormal proteins accumulate in tissues of individuals exposed to BMAA, and may lead to dysfunction. Interestingly, BMAA is incorporated into all cells, but only causes dysfunction in tissues with minimal cellular turnover, such as neurons, and retinal pigment epithelial cells. Abnormally folding proteins and subsequent protein aggregates are diluted amongst daughter cells in rapidly dividing tissues, leaving the majority of the body unaffected by dietary BMAA. Interestingly, a retinal dysfunction termed linear retinal pigment epitheliopathy, has been identified as a predictor of ALS in the Chamorro, and provides additional evidence of the link between BMAA and ALS development.
Since BMAA replaces L-serine during protein synthesis, L-Serine has been the subject of large scale clinical trials. It is hypothesized that high doses of L-serine may help in limiting the progression of the disease when used preventatively, which would be ground breaking for those affected by the disease.
For more information, and to donate to Dr. Paul Cox and his colleagues, please visit the Institute of EthnoMedicine, and Dr. Cox’s research associate’s page, Tellurian Toxins.
Thank you Dr. Kaleigh Anstett, Naturopath for providing the content for this article.
You are in my thoughts Don McCusker.
The Vitality Team