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Three independent laboratories recently identified immune organ defects in SMA. We therefore propose a novel pathogenic mechanism contributory to SMA, resulting in higher susceptibility to infection and exacerbated disease progression caused by neuroinflammation. Overall, compromised immune function could significantly affect survival and quality of life of SMA patients. We highlight the recent findings in immune organ defects, their potential consequences on patients, our understanding of neuroinflammation in SMA, and new research hypotheses in SMA pathogenesis.<\/jats:p>","DOI":"10.1002\/acn3.423","type":"journal-article","created":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T03:46:53Z","timestamp":1495079213000},"page":"522-530","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["New insights into SMA pathogenesis: immune dysfunction and neuroinflammation"],"prefix":"10.1002","volume":"4","author":[{"given":"Marc\u2010Olivier","family":"Deguise","sequence":"first","affiliation":[{"name":"Regenerative Medicine Program Ottawa Hospital Research Institute Ottawa Ontario K1H 8L6 Canada"},{"name":"Department of Cellular and Molecular Medicine University of Ottawa Ottawa Ontario K1H 8M5 Canada"},{"name":"Centre for Neuromuscular Disease University of Ottawa Ottawa Ontario K1H 8M5 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