Amyotrophic lateral sclerosis (ALS) is disease in which motor neurons in the brain degrade and eventually die. This leads to the loss of motor and muscle control resulting in muscle spasms, loss of motion in muscles, potential loss of speech, and even loss of respiratory function. This disease affects 20,000-30,000 people in the US with about 5,000 people being diagnosed each year. This disease progresses quickly and the average lifespan of a person diagnosed with ALS is roughly 3-5 years. The main cause of this disease is unknown, but there are genetic factors that make some people more likely to experience it than others. In this particular blog, I decided to go into a bit more detail about the “disorder of the week” for class since it became apparent in discussion that many people were quite uninformed about ALS prior to reading the assigned article.
The article of the week, Calcium-dependent protein folding in amyotrophic lateral sclerosis, sheds some light on how disturbances in calcium homeostasis and protein mis folding could be related to ALS. In general, dishomeostasis of calcium and mis folded proteins can lead to neurodegeneration. Specific proteins are found in cells in order to ensure that other proteins are folded correctly. The folding regulation proteins are usually regulated by calcium. This creates a distinct link between calcium levels and levels of mis folded proteins. Low calcium leads to low regulation of corrective proteins, which leads to high levels of mis folded proteins. Both of these events can lead to neurodegeneration.
The article tried to make a strong argument for how calcium levels and mis folded proteins can cause ALS. The article did a great job explaining how calcium is able to regulate the proteins that check for mis folded proteins in cells, but I don’t think it made a convincing argument for the cause of ALS. It did have some really great points, for example it explained why motor neurons are most affected by calcium. Motor neurons are more permeable/sensitive to calcium compared to sensory or other neurons. This makes their argument very strong to show why loss of muscular control would come from this imbalance of calcium and mis folded proteins. But in my opinion, explaining the motor neuron loss isn’t quite enough to prove that it is a cause of ALS. I think with further research into how this mechanism is related specifically to ALS and not just motor neuron loss in general would make the paper’s argument more convincing.
During the discussion of ALS in class, one interesting question came up about which would be worse: To have a neurological disorder that leave you cognitively impaired but physically fine, or one which leave you cognitively functioning but physically very ill. This question really made me think. I thought instantly about Alzheimer’s disease as it would compare to ALS. I personally have never known anyone with ALS, but I have seen AD in multiple people. Both diseases are extremely hard for both the patient and the family, but difficult in what seems to be different ways. I think it would be incredibly frustrating to be cognitively functioning normally but unable to control my body movements or speech like an ALS patient. It would also be frustrating to be physically functioning fine, but be afraid or confused by my own family members due to the memory loss associated with AD. If I had to choose one of these diseases, I think I would prefer to be physically functioning normally but cognitively dysfunctional. I think losing the ability to perform simple tasks on my own and have control of my body would too difficult, especially if I was able to comprehend and perceive the world in the same was as myself “pre ALS.”
This specific disease made me think about neurodegeneration in a new way. Previously, most of the diseases we discussed in class had to do with cognitive decline due to neurodengeration. Even the topic of obesity led to cognitive decline, which to me wasn’t an obvious connection. I think the fact that the brains of ALS patients remain able to think and learn correctly even when their motor neurons are have experienced extreme damage. It is quite interesting how the motor neurons seem to be the only ones effected in ALS, but that seems to be explained by dysregulation of calcium which leads to the buildup of mis folded proteins in the brain. Usually in my blogs I end with some sort of suggestion as to what can be done to prevent the “disease of the week.” Unfortunately this week I don’t have much advice since the exact cause of ALS is unknown. But as usual, everything in moderation will help keep a natural balance in your body.
