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Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease1. The pathology of ALS is described as the progressive degeneration of motor neurons that initially leads to atrophy of the voluntary muscles followed by the involuntary muscles1,2. Ultimately, the cause of death is pulmonary distress due to loss of function of the diaphragm2. Life expectancy after diagnosis is usually one year, and there are currently no cures or effective treatments for this fatal disease3. Approximately 90% of all ALS cases are sporadic meaning that the disease is developed randomly, while around 10% of the cases are familial meaning that the disease is passed down within a family3,4. Over 30 years, many genes have been identified and linked to the development of familial ALS. One of these genes is MATR3 which codes for Matrin-3, a nuclear matrix protein that binds to DNA and RNA with various roles in the nucleus5. Matrin-3 is normally found in the nucleus; however, when the gene is mutated, Matrin-3 is depleted from the nucleus and accumulates in clusters in the cytoplasm6. Matrin-3 associated toxicity is hypothesized to be either due to the loss of function of the protein in the nucleus or a gain of toxicity function in the cytoplasm leading to neuronal cell death. Furthermore, with increasing MATR3 toxicity in yeast, an increase in cell size was observed.

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