ALS – The Science Behind the Disease

Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig’s Disease, is a fatal neurodegenerative disorder that results in the withering away of muscles due to the malfunction of motor neurons that travel from the spinal cord to the various skeletal muscles in the body. The lateral part of the spinal cord containing these motor neurons develops “sclerosis” where it hardens due to certain glial cells conglomerating together and essentially forming a scar in the spinal cord. This disrupts the signal that the motor neurons bring to the muscles, and eventually results in the muscles not being able to contract and “atrophying,” or withering away.
There has been a recent surge in the recognition of ALS with the “Ice Bucket Challege” that I’m sure most of us either heard about or participated in. ALS is a very debilitating disease that results in requiring a wheelchair to get around and eventual death. At this point there is no cure, and I’m not sure that a true “cure” will ever be developed. However, the recent advancements in scientific research are beginning to show us the possible root cause of this disease and possible treatments to help prevent and delay the development and progression of this debilitating disease.
There are receptors on neurons throughout your body called NMDA receptors. They are usually found with another receptor called AMPA. These are both responsive to a neurotransmitter called glutamate. In ALS, and other nuerodegenerative diseases, there is too much glutamate causing these receptors to activate, letting a lot of calcium into your cells. This calcium puts stress on the organelles inside the cell, especially the mitochondria and the endoplasmic reticulum (ER). This stress signals cell death in many cells. It also causes the ER of cells still alive to not fold proteins correctly; a specific misfolded protein called SOD1 is thought to play a major role in ALS because it leads to the accumulation of Reactive Oxygen Species (ROS) that can cause a lot of damage within cells. Glial cells help out neurons, and in this case they surround the damaged motor neurons to help prevent further damage. This is what results in the hardend scars found in ALS. Scientists are still uncertain to why the motor neurons in the spinal cord are the only cells that are affected in ALS. It may have to do with the type and amount of NMDA receptors found in motor neurons, but this is still unclear.
At any rate, this further understanding had lead to new developments within the treatment of ALS. NMDA receptor blockers may potentially be able to alleviate some of the effects of ALS. This is tricky however, because these receptors are found elsewhere in the body and their normal healthy function is necessary. Also, antioxidants, even like those found in grapes and blueberries, could also help stop some of the damage caused by the ROS. The best bet is a combination of NMDA antagonists, antioxidants, and cell stress reduction through a healthy lifestyle. More research is necessary to understand the full mechanism of this disease, which is essential in creating the most efficient treatment plan. Scientists are on their way to cracking the code of ALS, but they haven’t quite found it yet.