Amyotrophic Lateral Sclerosis: A Degenerative Disease

By Armand Zeiders


There are many different types of degenerative diseases. With Amyotrophic Lateral Sclerosis, patients suffer from motor neuron degeneration, and there are about 25,000 individuals in the United States that have ALS, which also is known often as Lou Gehrig's disease after the famous New York Yankee whose career and life were cut short by ALS.

The motor neurons that are affected when one has Amyotrophic Lateral Sclerosis or ALS are part of every human's central nervous system. While there are several similar types of motor neuron diseases, ALS is the most prevalent. Generally, people begin to experience symptoms of ALS around the age of 40, with typical onset being between 40 and 60 years of age. However, there have been cases of much younger individuals contracting the disease. Unfortunately, there is no current cure for this fatal disease, and death typically occurs in less than three years after the onset of symptoms. Occasionally, a patient will live ten or more years with ALS, but this is very rare.

Because the neurons begin to degenerate, this causes muscles to begin wasting away, and the first symptoms are initially a general weakening of the muscles. Often a person will find that a previously fluid movement becomes jerky and difficult, such as swinging a tennis racket. The person might begin to fumble when completing normal tasks, such as buttoning a garment or brushing hair. Stumbling and dragging a limb while walking also can occur, as well as a slurring of speech or a change in vocal tone.

As time passes, the degeneration can cause a person to lose most of their ability to move, and most end-stage ALS patients are confined to wheelchairs or even bed-ridden and hospice care is needed. Difficulty swallowing can cause those with ALS to choke and become unable to eat solid foods, and loss of respiratory function also often occurs. Patients often are fed through a tube and placed on ventilators to ensure nutrition and breathing. There are a few drugs that can help ease some of the symptoms, and one drug has been developed that slows the progression of the disease slightly, but no truly effective treatments have been created yet.

Scientists are not certain what causes ALS, however, scientists did identify a gene mutation that was related to about 20 percent of cases. In addition, researchers have identified a chromosome that seems to be a factor in familial or inherited ALS. In 2011, scientists studying the protein C9orf72 found a link between mutations in this protein and amyotrophic lateral sclerosis. In a person without ALS, sections of this protein repeat about 30 times, but in a person with the mutation, this repeat occurs several hundred times. While scientists do not know yet why the mutation occurs, many medical researchers believe this problem is a direct cause of ALS.

There are many diseases that are linked to problems with protein and in addition to ALS. These diseases include Alzheimer's disease and Parkinson's diseases. All of these diseases are highly debilitating and eventually fatal; therefore, so much study is concentrated on understanding the causes and development treatments or cures. To assist in this important work, biotechnology companies offer important services such as protein sequencing, protein synthesis and peptide synthesis. Hopefully, these scientists will find a way to eliminate these diseases or at least treat them and prolong the patient's enjoyment of life as long as possible.




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