Monday, 5 August 2013

Information About Amyotrophic Lateral Sclerosis

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.

Often the initial symptom of ALS is muscle weakness, and this is because when the neurons begin to degenerate our muscles atrophy. Often a person with early stage ALS will experience weakness as well as not being able to control typical involuntary movements, such as lifting a cup of coffee or throwing a ball. Muscle twitching is another early symptom, as well as slurred speech, difficulty walking or running and problems with normal tasks such as writing, brushing teeth or buttoning a shirt.

Eventually, as the disease progresses, sufferers often have trouble moving at all and are confined to a wheelchair. Difficulty in speaking can become progressively worse, and difficulty swallowing also occurs as the disease progresses. Respiratory problems also occur in later stages, and those with ALS often are put on long-term automatic ventilators to help them breath. While there are some drugs available to help ease the symptoms, only one drug helps to slow progression of the disease, but typically survival rates are only increased by a matter of months to a few years.

Medical researchers to do not know exactly what causes this disease, but in the early 1990s, a mutation in a specific gene was found to have a link with 20% of ALS cases. Just a few years ago, scientists also discovered a link between the protein known as C9orf72 and ALS. This protein is mutated in those that have ALS, and a certain section of the protein sequence repeats over and over again. In most people this section repeats about 30 times, but with an ALS patient, it repeats hundreds of time. Of course, researchers do not know what causes the mutation; they just know that it seems to cause the disease. In addition, while inherited ALS is rare, scientists did discover a chromosomal problem that is prevalent in inherited 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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