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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2016,12,20]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Clinical and pathological hallmarks shared by various familial and sporadic forms of amyotrophic lateral sclerosis (ALS) suggest common underlying mechanisms of disease. Using a series of ALS mouse models, we demonstrate that one shared feature of ALS is the selective sparing of gamma motor neurons (\u03b3-MNs), which innervate muscle spindles and regulate primary proprioceptive afferent (I\n                    <jats:sub>A<\/jats:sub>\n                    ) feedback on alpha motor neurons (\u03b1-MNs). Genetic evidence presented here implicates this major excitatory input in the selective degeneration of \u03b1-MNs in ALS. Functional elimination of I\n                    <jats:sub>A<\/jats:sub>\n                    inputs or partial elimination of \u03b3-MNs is protective in superoxide dismutase-1 (SOD1) mutant mice, suggesting that surviving \u03b3-MNs contribute to \u03b1-MN loss by increasing muscle afferent-mediated excitation. This study highlights the role of synaptic connectivity and circuit function in motor neuron disease.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1605210113","type":"journal-article","created":{"date-parts":[[2016,12,7]],"date-time":"2016-12-07T22:30:25Z","timestamp":1481149825000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":109,"title":["Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS"],"prefix":"10.1073","volume":"113","author":[{"given":"Melanie","family":"Lalancette-Hebert","sequence":"first","affiliation":[{"name":"Center for Motor Neuron Biology and Disease, Columbia University, New York, NY 10032;"},{"name":"Department of Neurology, Columbia University, New York, NY 10032"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aarti","family":"Sharma","sequence":"additional","affiliation":[{"name":"Center for Motor Neuron Biology and Disease, Columbia University, New York, NY 10032;"},{"name":"Department of Neurology, Columbia University, New York, NY 10032"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander K.","family":"Lyashchenko","sequence":"additional","affiliation":[{"name":"Center for Motor Neuron Biology and Disease, Columbia University, New York, NY 10032;"},{"name":"Department of Neurology, Columbia University, New York, NY 10032"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil A.","family":"Shneider","sequence":"additional","affiliation":[{"name":"Center for Motor Neuron Biology and Disease, Columbia University, New York, NY 10032;"},{"name":"Department of Neurology, Columbia University, New York, NY 10032"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2016,12,7]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrn3430"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880180808"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2009.10.017"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410040309"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.051508.135722"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0012162200000116"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00688606"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-198007000-00004"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880130612"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.20-07-02534.2000"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2011.06.031"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/nn1653"},{"key":"e_1_3_3_13_2","first-page":"33","article-title":"Structural organization of the extraocular muscles","volume":"2","author":"Spencer RF","year":"1988","unstructured":"RF Spencer, JD Porter, Structural organization of the extraocular muscles. 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