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Loss of\n                    <jats:italic>\n                      <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\n                      1\n                    <\/jats:italic>\n                    leads to a dramatic reduction in\n                    <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\n                    protein, which is essential for motor neuron survival.\n                    <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                    disease severity ranges from extremely severe to a relatively mild adult onset form of proximal muscle atrophy. Severe\n                    <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                    patients typically die mostly within months or a few years as a consequence of respiratory insufficiency and bulbar paralysis.\n                    <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                    is widely known as a motor neuron disease; however, there are numerous clinical reports indicating the involvement of additional peripheral organs contributing to the complete picture of the disease in severe cases. In this review, we have compiled clinical and experimental reports that demonstrate the association between the loss of\n                    <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\n                    and peripheral organ deficiency and malfunction. Whether defective peripheral organs are a consequence of neuronal damage\/muscle atrophy or a direct result of\n                    <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\n                    loss will be discussed.\n                  <\/jats:p>","DOI":"10.1111\/joa.12083","type":"journal-article","created":{"date-parts":[[2013,7,23]],"date-time":"2013-07-23T00:46:14Z","timestamp":1374540374000},"page":"15-28","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":233,"title":["Spinal muscular atrophy: a motor neuron disorder or a multi\u2010organ disease?"],"prefix":"10.1111","volume":"224","author":[{"given":"Monir","family":"Shababi","sequence":"first","affiliation":[{"name":"Department of Veterinary Pathobiology Life Sciences Center University of Missouri Columbia MO USA"},{"name":"Department of Molecular Microbiology and Immunology School of Medicine University of Missouri Columbia MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian L.","family":"Lorson","sequence":"additional","affiliation":[{"name":"Department of Veterinary Pathobiology Life Sciences Center University of Missouri Columbia MO USA"},{"name":"Department of Molecular Microbiology and Immunology School of Medicine University of Missouri Columbia MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine S.","family":"Rudnik\u2010Sch\u00f6neborn","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Medical Faculty University Hospital Aachen Aachen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,7,22]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.01.071"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.3700163"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1993.tb02395.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI29562"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PHM.0b013e31804b1d66"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PHM.0b013e31804a8505"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.11.1961"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9568.1999.00428.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/mt.2012.261"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq300"},{"key":"e_1_2_7_12_1","first-page":"CD006282","article-title":"Drug treatment for spinal muscular atrophy types II and III","volume":"1","author":"Bosboom WM","year":"2009","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr067"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12071"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq021"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2011.09.007"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1741-7015-10-24"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23582"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)91439-0"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/344540a0"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)92206-7"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/7.12.1927"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-009-0012-x"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2009.11.148"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg157"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.4155\/fmc.12.107"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.152.5.1107"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.12.1439"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/0167-5273(89)90005-3"},{"key":"e_1_2_7_30_1","first-page":"119","article-title":"[Type I spinal atrophy (Werdnig\u2010Hoffman disease). 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