Amyotrophic Lateral Sclerosis
University of Southern Mississippi
Dr. Jennifer Walker
BSC 370 Genetics, Spring 2010
Imagine being not able to move your muscles freely everyday, and that process has to coordinate between the brain and the spinal cord to tell the muscles to move. However, when this coordination is challenged and can not convey signals, the results are no muscle movement. At first, muscles in the hands, arms, and legs feel weak. The hands and feet twitch. Speaking and eventually breathing become difficult, and paralysis occurs in the final stages, although thinking and other cognitive functions remain intact. Death usually occurs within five years of the onset of symptoms. This rare, progressive disorder is also known as Lou Gehrig’s disease, after a famous baseball player whose life was cut short by it. What is Amyotrophic Lateral Sclerosis? Nowadays, more and more persons are being detected with a condition that is signified as “Amyotrophic Lateral Sclerosis”. In the history, this neurological situation was signified by several as “Lou Gehrig’s disorder” (Hohnsbeen, 2008). Those that suffer from this medical condition endure great complexities. This is essentially because of the fact that the disorder quickly wages war against some nerve cells in the body. These cells are recognized as neurons by medical experts. The neurons that are alluring to the disorder are the ones that are absolutely liable for the control of muscles that are deemed to be voluntary. The muscles in the upper body as well as those in the lower body are influenced. The influenced neurons are situated in the brain as well as the spinal cord and finally die (Hohnsbeen, 2008).
In this direction, one will learn the facts adjacent the neurological state of amyotrophic lateral sclerosis. When a person is detected with amyotrophic lateral sclerosis, the advancement of the disorder may be gradual or it may be rapid. It all depends on the general health of the individual, how long they have had the condition previous to diagnosis, plus any other medical circumstances that they may endure from (Phillies, 1995). Seeing that the reason of this severe neurological condition is unidentified, medical experts often have a hard time deciding the speed in which the condition improvements. Nevertheless, it is a fact that it will improve and signs observed will become more demanding to the functionality of the body. Difficulties that engage the muscles of the body that are utilized for different purposes will be experienced (Phillies, 1995). It is not common for the amyotrophic lateral sclerosis sufferer to experience difficulties such as problems in walking, situations in which it is challenging to eat and swallow as suitable, and several may even face severe medical emergencies such as the failure to breathe properly. The cause of amyotrophic lateral sclerosis is not identified, though a significant step toward deciding the cause came in 1993 when scientists found that changes in the gene that generates the Cu/Zn superoxide dismutase (SOD1) enzyme were related with some cases (about twenty-percent) of familial Amyotrophic lateral sclerosis.
This enzyme is an influential antioxidant that defends the body from harm caused by superoxide, a toxic free radical produced in the mitochondria (William, 2000). Free radicals are very reactive molecules made by cells during normal metabolism again mostly by the mitochondria. Free radicals can build up and cause harm to both mitochondrial and nuclear DNA and proteins within cells. Proof proposes that inability of defenses against oxidative pressure up-regulates programmed cell death (apoptosis), among several other potential results. Although it is not yet obvious how the SOD1 gene mutation leads to motor neuron degeneration, examiners have theorized that an accumulation of free radicals may consequence from the faulty functioning of this...
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