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Introduction Amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, is a

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Introduction
Amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, is a neurodegenerative disease. Originally acknowledged by Jean-Marie Charcot in 1874, this disease kills over 140,000 people worldwide every year. The disease causes progressive paralysis and muscle weakness due to destruction of both upper and lower motor neurons. Due to its insidious nature and the irreversible negative effects on skeletal muscle innervation, ALS has a survival duration time of only 3 to 5 years (Rowland).

Etiology & Incidence The cause of ALS is unknown. No consistent links have been found between ALS and race, ethnicity, or location. Men appear to be more likely to have ALS, and older people between the ages of 40 and 70 have it more …show more content…

When family trees were assembled for the inhabitants who had ALS, it was found that direct relatives of the inhabitants were at significantly increased risk for developing the disease themselves as opposed to the inhabitants who had no relatives with ALS. This incidence seemed to imply there was a strong genetic factor playing a role in ALS development (Plato). There are several factors being explored as potential causes. Because there are familial cases of ALS, gene-related causes are worthy of investigation with the SOD1 (copper-zinc superoxide dismutase) gene being one of greater interest. Mutations in the SOD1 gene have been found in a fifth of familial cases of ALS (Rosen). Mutations to this gene, rather than having a detrimental effect on its own antioxidant enzyme function, appear to cause disease through toxic gain (Shaw). Oxidative stress and increase in reactive oxygen species can cause cell death, and because mutations in the SOD1 gene can allow this to occur, it could be a potential mechanism for ALS neural degeneration (Wijesk).
Another genetic influence could be mutations in mitochondrial DNA. The mutated DNA causes mitochondria to malfunction. Elevated calcium levels and decreased energy production in the mitochondria have been documented in patients with SALS (Wiedemann).
Another possible cause of ALS is over-excitability of motor neuron glutamate receptors. Repeated stimulation of these receptors

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