Synonyms
Historical Background
Amyotrophic lateral sclerosis (ALS) is a heterogeneous neurodegenerative disease that leads to progressive muscle deterioration and commonly death within 3–5 years of symptom onset. The first gene established to cause ALS when mutated was SOD1, identified by a team of geneticists in 1990. Since then, more than 25 genes have been identified or implicated in ALS (Marangi and Traynor 2015). Though awareness of the genetic factors that cause ALS is impressive, clear understanding of the mechanism or mechanisms by which the known mutations trigger motor neuron degeneration is lacking. Several hypotheses have been proposed including protein aggregation, defective RNA processing, oxidative stress, subcellular organelle dysfunction, increased apoptosis, neuroinflammation, and glutamate excitotoxicity (Paez-Colsante et al. 2015). All are supported by evidence indicating there are many different potential ways by which motor neuron function can be...
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Martin, A.P., Topp, J.D. (2018). ALS2. In: Choi, S. (eds) Encyclopedia of Signaling Molecules. Springer, Cham. https://doi.org/10.1007/978-3-319-67199-4_101505
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DOI: https://doi.org/10.1007/978-3-319-67199-4_101505
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