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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.The onset of amyotrophic lateral sclerosis.
de Carvalho M, Swash M.
J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):388-9.
2.Genetics of familial and sporadic amyotrophic lateral sclerosis.
Gros-Louis F, Gaspar C, Rouleau GA.
Biochim Biophys Acta. 2006 Feb 10
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