Amyotrophic Lateral Sclerosis (ALS) is also known as Lou Gehrig’s disease. It is an invariably fatal neurological disease that attacks the nerve cells (neurons) that control voluntary …show more content…
This process involves oxygen in the break down of glucose, resulting in the production of two important free radicals – superoxide radical and hydroxyl radical (Bogdanov et al., 1998). Among these two, the hydroxyl radical is the most potent one; however, if these radicals are not broken down into pieces, then they will accumulate in high concentrations and will eventually begin to kill cells. There is no clear cut pathophysiology known yet, but there are possible mechanisms that are at the root of the pathophysiology of ALS. Some of these include: (1) the oxidative damage on cells by SOD1 (superoxide dismutase), and (2) increased glutamate concentration in the cerebrospinal fluid …show more content…
In this study, it was found that ALS patients have a deficiency of glutamate transporter proteins (Watanabe et al., 2001). These proteins carry free glutamate into surrounding astrocytes, a star-shaped neuralgia cell of the nervous tissue. Normally, free glutamate would be directly removed (Fig. 2). In their research, a key protein called glutamate transporter 1 (GLT-1) is found to be deficient in the brains and spinal cords of some patients who had died of ALS. GLT-1 is an important protein for it clears away excess glutamate. Rothstein (2009) added in their research that the damage caused by the loss of GLT-1 is not happening all at once. According to their study, when a glutamate transport blocker is introduced into a spinal cord, the glutamate levels increased tremendously. This lasts for week. After the first few weeks, neurons appeared to have been normal slowly die (Rothstein, 2009). In the following weeks, patients with ALS who are lacking the glutamate transporter protein experience a rise in glutamate and aspartate within the spinal cord. Ultimately, high concentrations of both glutamate and aspartate destroy the large motor neurons in the spinal