Amyotrophic Lateral Sclerosis (ALS) is more commonly known as Lou Gehrig’s disease after the New York Yankee baseball player Lou Gehrig. During his season in 1938, Lou Gehrig started to experience muscle weakness which started to affect his batting and even his ability to tie his shoes. A diagnosis of ALS was soon made, bringing this otherwise not well known disease into the public’s eye.
ALS is characterized by muscle weakness or cramps, cognitive impairments, slurred speech, or difficulty swallowing. The disease often appears with similar symptoms to those of Parkinson’s disease or even Alzheimer’s disease, making the diagnosis of this disease based mainly on ruling out other diseases.
ALS is a degenerative disease that causes death in motor neurons of the brain, spinal cord, and nerves running to muscles. Motor neurons signal movement to occur in muscles by sending a signal from the brain to peripheral muscles. Death to neurons is caused genetics and oxidative stress on the cell.
One of the most important ideas to understanding the science behind ALS is to understand the role of the mitochondria in the cell. The mitochondria are the powerhouse of the cell. It helps to break down molecules so that the cell can use them for energy.
When the mitochondria become overworked, this leads to mitochondrial stress. During mitochondrial stress, extra molecules called oxidants are produced that are harmful to the cell. In response to excessive amounts of oxidants, the cell turns on pathways that signal the cell to die.
Oxidants are the cause of motor neuron death.
There are a few ways to get rid of oxidants. The way that the body normally uses is the SOD1 protein which breaks down oxidants into less harmful substances. Another way is by activation of the OXR1 gene which is the bodies natural response to oxidants. The last way to get rid of oxidants is through antioxidants which protect the mitochondria against oxidative stress.
All of these methods of getting rid of oxidants are currently being used in research to try to slow down the progression of ALS. There are currently no existing treatments for ALS that target oxidants as it can be very hard to target the exact motor neurons where the extra oxidants are forming. Future research will continue to look into oxidants as well as stem cells, optogenetics, and other medications.
Although Lou Gehrig might have been one of the first public cases of ALS, there have been many more cases and much more research conducted since his death. Lou Gehrig died from ALS, but his advocacy for the disease has lead to increased amounts of research, and promise for more effective treatment around ALS.
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