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Pathogenesis of ALS

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ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig’s disease, is a devastating neurodegenerative disorder, which is characterized by the selective degeneration of upper and lower motor neurons, the large nerve cells connecting the brain to the spinal cord and from the spinal cord to muscles, which control muscle movement. The loss of motor neurons leads to progressive atrophy of skeletal muscles. ALS is a relentless disease that manifests as progressive decline in muscular function resulting in eventual paralysis, speech deficits and, ultimately, death due to respiratory failure in the majority of ALS patients within 2 to 5 years of clinical onset. In spite of its notoriety, the mechanisms underlying ALS remain obscure and therapies with long-term benefit are lacking. [...]


1.Amyotrophic Lateral Sclerosis: New Perpectives and Update.
Orsini M, Oliveira AB, Nascimento OJ, Reis CH, Leite MA, de Souza JA, Pupe C, de Souza OG, Bastos VH, de Freitas MR, Teixeira S, Bruno C, Davidovich E, Smidt B.
Neurol Int. 2015 Sep 24;7(2):5885. doi: 10.4081/ni.2015.5885. eCollection 2015 Sep 24. Review.
2.ALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium Triad.
Kaus A, Sareen D.
Front Cell Neurosci. 2015 Nov 19;9:448. doi: 10.3389/fncel.2015.00448. eCollection 2015. Review.
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