This past week the Fargo-Moorhead community lost an angel to a disease that continually seems to rip away our hope and block any light at the end of the tunnel. This one, like many diseases does not discriminate against who it takes. ALS, amyotrophic lateral sclerosis, is considered a debilitating and imprisoning disease with no cure. Little hope is found in those words. Therefore, we ask: What can we do as a community, as people who love each other, to help those who suffer from ALS, along with their families? What good can we bring of such hurt?
First, let’s begin with a brief overview of the disease from a biological standpoint to better understand its complexity.
ALS is a fatal disease that is commonly associated with the degeneration of upper and lower motor neurons, or the cells that allow our muscles to move. It can be a genetic disease, running in families, or randomly occurring. In the body of someone affected by ALS, there are essentially three things that are going wrong and leading to the disease.
The first thing going wrong is oxidative stress and RNA dysmetabolism, both of which are a part of a cycle that essentially leads to magnifying each other. Oxidative stress is a result of natural metabolic pathways that are working in overdrive and their products accumulate. These oxidative products cause damage to the cell and other cellular changes that are detrimental to normal function. In ALS, oxidative stress causes RNA-binding proteins TDP43 and FUS to delocalize from their neutral location and aggregate in the cell. This then hinders the proteins’ ability to function properly and metabolize RNA. Oxidative stress also allows for the production and release of stress granules which allow for the activation of “stress genes” and are characteristic of ALS neurodegeneration.
When RNA is unable to be properly metabolized, proteins such as FUS and TDP43 can create a negative impact on natural oxidative stress protections. This is the fatal cell cycle that continues to occur in ALS patients.
The second malfunction is protein folding. All the oxidative stress occurring in the cells leads to mutated proteins which aggregate and trap helper proteins that act to remove malfunctioning proteins. The cell is then unable to respond to the damage occurring, so it naturally performs autophagy which is to eat or destroy itself.
The third component that is failing in ALS is metal homeostasis. In patients suffering from ALS, there is an unusual metabolism of iron and copper which leads to an increase in both. This then leads to oxidative stress, which was previously discussed, which causes overall protein degradation or cell death.
As you can see, ALS is incredibly complex and gene specific in its effects. There is no clear pathway but rather a culmination of many pathways working in tandem. Because much of the biological pathway of ALS is still unclear, more research is required. As of now, the only treatment options available treat solely the symptoms. Therefore, the more we can learn, even at a small scale, can help add to the knowledge of specialized treatment options.
So, what does all this complexity and uncertainty mean to us and to our community?
It means there is much work and awareness that needs to be done. It means we come together as a community and lift each other up in kindness. It means we turn to faith for hope and we rely on the love of our faith to radiate and bring meaning to people’s lives. Finally, it means we do not turn our back on those suffering but be friends and be of service to our community’s needs.
Right now, it feels like there is no hope. However, with faith, kindness, love and many more hours of hard work and research, there can be immense hope.
“But those who hope in the Lord will renew their strength. They will soar on wings like eagles; they will run and not grow weary, they will walk and not be faint.” Isaiah 40:31.
https://www.ncbi.nlm.nih.gov/pubmed/27150074
ALS – Finding Hope
