If I say the words “ice bucket challenge”, do you know what purpose of it is? Or what it is aiming to cure? The answer is the all-too-familiar Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease. ALS has been on the back-burner of recent research mainly due to the fact that it affects only 30,000 Americans today. This is in comparison to the millions of people with some form of cancer, which respectfully has been a forefront of research for decades. In recent years, ALS has captivated copious amounts of attention. We may know that finding the cure to ALS is the purpose of the ice bucket challenge, but what is ALS exactly? Although I participated in an ice-cold bath to raise money, I struggled with that very question.
ALS is a neurodegenerative disease that is physically characterized by a decrease in motor function which is an effect of a loss of motor neurons in the brain stem, spinal cord and cerebral cortex. Although the reason for loss of function in motor neurons is still under investigation, glutamate toxicity is a main suspect.
At the end of each neuron, where glutamate is released to the receiving neuron, there is a little space called the synaptic cleft housing enzymes that will terminate the glutamate action or cause it to be a factor of reuptake for use again, and to prevent over-accumulation of glutamate. If there is excessive glutamate in this cleft and/or a dysfunction of one or more of these enzymes, glutamate’s receptors may be over-activated which would lead to the undesired massive influx of calcium with the end result being toxic to the neuron. There is also the possibility that one of the glutamate receptors, NMDAR, is potentially seen to be over-activated in ALS patients. Why is this important? Well, since NMDAR is permeable to calcium, and if high calcium levels may result in neuronal death, over-activation may play a vital role in what characteristics of ALS we are seeing. Although the process is more complicated than that, this is a major area of focus.
So what can we do so far? We know that excessive levels of glutamate in aforementioned clefts were a bad thing, so how can we prevent that? Today, there is a drug, called Riluzole, that remains the only disease-modifying drug for treatment of ALS. It does so by controlling by reducing glutamate release, uptake and glutamate receptor function. Although it hasn’t had significant improvements on patient lives (only extending ventilator-free living for a couple months for roughly $10 a pill), it still is a step in the right direction.
With more research, like all diseases, a better way can be found. Although a minority of the population is being affected, it is roughly 15 new diagnoses in the United States alone. That is 15 new families impacted by this debilitating disease which could be prevented if this research were carried out farther.
The beginning of the cure for ALS.
