Amyotrophic Lateral Sclerosis (ALS): Lou Gehrig's Disease Essays

Amyotrophic Lateral Sclerosis (ALS): Lou Gehrig's Disease Essays

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Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease, affects approximately 20,000 - 30,000 U.S. citizens, with an estimated 5,000 new diagnoses made each year. ALS is a rapidly progressive neurological disease which attacks neurons responsible for the control of voluntary muscles, and is habitually fatal. ALS is part of the class of disorders called motor neuron diseases (MNDs). Motor neuron diseases are characterized by the gradual degeneration of motor neurons, ultimately leading to the death of affected motor neurons.
Motor neurons are defined as nerve cells located in the central nervous system (CNS) whose axons project outside of the CNS to either directly, or indirectly control muscles. Motor neurons are efferent nerves, which carry signals to muscles from the spinal cord to produce a movement (i.e. contraction). Motor neurons can be categorized as either upper or lower. Upper motor neurons travel down the spinal cord from the CNS, where they synapse with lower motor neurons which travel from the spinal cord to specific motor units in muscle. The degeneration of motor neurons caused by ALS affects both the upper and lower motor neurons, causing the neurons to die or degenerate progressively until they are no longer capable of transmitting messages to the muscle to induce contraction. Atrophy of muscle, along with the development of fasciculations (fine twitches), in subjects with ALS, is attributed with the aforementioned inability to properly function.
Subjects diagnosed with ALS are given a general life expectancy of roughly three to five years, after the onset of symptoms, due to the accelerated deterioration of the body; however, an estimated 10% of individuals with ALS survive for 10 or more y...

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... J., Koppers, M., Berg, L. H., & Pasterkamp, R. J. (2013). Protein aggregation in amyotrophic lateral sclerosis. Acta Neuropathologica,125(6), 777-794.
[5] #Mackness, B., Tran, M., McClain, S., Matthews, R., & Zitzewitz, J. (2014). Folding of the RNA REcognition Motif (RRM) Domains of the Amyotrophic Lateral Sclerosis (ALS)-linked Protein TDP-43 Reveals an Intermediate State.The Journal Of Biological Chemistry,289(12), 8264-8276.
[6] #Gerber, Y. N., Privat, A., & Perrin, F. E. (2013). Gacyclidine improves the survival and reduces motor deficits in a mouse model of amyotrophic lateral sclerosis.Frontiers in cellular neuroscience, 7(280), N/A.
[7] #Castillo, K., Nassif, M., Valenzuela, V., Rojas, F., Matus, S., Mercado, G., et al. (2013). Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons.Autophagy, 9(9), 1308-1320.

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