{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:14:25Z","timestamp":1791296065297,"version":"4.1.0"},"reference-count":58,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2012,4,2]],"date-time":"2012-04-02T00:00:00Z","timestamp":1333324800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Comparative Neurology"],"published-print":{"date-parts":[[2012,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Feline spinal muscular atrophy (SMA) is a fully penetrant, autosomal recessive lower motor neuron disease in domestic cats that clinically resembles human SMA Type III. A whole genome linkage scan identified a \u223c140\u2010kb deletion that abrogates expression of <jats:italic>LIX1<\/jats:italic>, a novel SMA candidate gene of unknown function. To characterize the progression of feline SMA, we assessed pathological changes in muscle and spinal cord from 3 days of age to beyond onset of clinical signs. Electromyographic (EMG) analysis indicating denervation occurred between 10 and 12 weeks, with the first neurological signs occurring at the same time. Compound motor action potential (CMAP) amplitudes were significantly reduced in the soleus and extensor carpi radialis muscles at 8\u201311 weeks. Quadriceps femoris muscle fibers from affected cats appeared smaller at 10 weeks; by 12 weeks atrophic fibers were more prevalent than in age\u2010matched controls. In affected cats, significant loss of L5 ventral root axons was observed at 12 weeks. By 21 weeks of age, affected cats had 40% fewer L5 motor axons than normal. There was no significant difference in total L5 soma number, even at 21 weeks; thus degeneration begins distal to the cell body and proceeds retrogradely. Morphometric analysis of L5 ventral roots and horns revealed that 4 weeks prior to axon loss, motor axons in affected cats failed to undergo radial enlargement, suggesting a role for the putative disease gene <jats:italic>LIX1<\/jats:italic> in radial growth of axons. J. Comp. Neurol. 520:1737\u20131750, 2012. \u00a9 2011 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/cne.23010","type":"journal-article","created":{"date-parts":[[2011,11,25]],"date-time":"2011-11-25T07:10:43Z","timestamp":1322205043000},"page":"1737-1750","source":"Crossref","is-referenced-by-count":6,"title":["Failure of lower motor neuron radial outgrowth precedes retrograde degeneration in a feline model of spinal muscular atrophy"],"prefix":"10.1002","volume":"520","author":[{"given":"Erin N.","family":"Wakeling","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9atrice","family":"Joussemet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Costiou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Fanuel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Moullier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Barkats","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Fyfe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,4,2]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ar.1090940210"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-003-0743-9"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/93.1.15"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-009-0012-x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1961.00450140022003"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.12.1439"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1006\/nbdi.1996.0010"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0195-5616(03)00079-2"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2002.00927.x"},{"key":"e_1_2_6_11_1","first-page":"2","volume-title":"Clinical neurology in small animals\u2014localization, diagnosis and treatment","author":"De Lahunta A","year":"2001"},{"key":"e_1_2_6_12_1","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1136\/jmg.8.4.481","article-title":"The nosology of the spinal muscular atrophies","volume":"4","author":"Emery AE","year":"1971","journal-title":"J Med Genet"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-200402090-00013"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1101\/gr.5268806"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200308159"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3765-08.2009"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00689549"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1090289"},{"key":"e_1_2_6_19_1","first-page":"1157","article-title":"Spinal muscular atrophy: foetal\u2010like histopathological pattern in Werdig\u2010Hoffman disease","volume":"50","author":"Hausmanowa\u2010Petrusewicz I","year":"1974","journal-title":"Bull N Y Acad Med"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.20453"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000153625.46892.6F"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.84.10.3472"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.101.4.1332"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.14-11-06392.1994"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M202037200"},{"key":"e_1_2_6_26_1","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1001\/archneurpsyc.1956.02330230050005","article-title":"Heredofamilial juvenile muscular atrophy simulating muscular dystrophy","volume":"75","author":"Kugelberg E","year":"1956","journal-title":"AMA Arch Neurol Psychiatry"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2008.00910.x"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.ne.19.030196.001155"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00688247"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.2.259"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.11.6307"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.22.18.6533-6541.2002"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2010.01.006"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1567-133X(02)00018-2"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.7.1177"},{"key":"e_1_2_6_37_1","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1083\/jcb.200208079","article-title":"A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy","volume":"160","author":"Monani UR","year":"2003","journal-title":"Cell"},{"key":"e_1_2_6_38_1","first-page":"974","article-title":"Motor nerve conduction velocity in spinal muscular atrophy of childhood","volume":"51","author":"Moosa A","year":"1976","journal-title":"BMJ"},{"key":"e_1_2_6_39_1","first-page":"423","article-title":"International SMA consortium meeting","volume":"2","author":"Munsat TL","year":"1992","journal-title":"Neuromuscul Disord"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00293462"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm367"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81632-3"},{"key":"e_1_2_6_43_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1074962"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880010110"},{"key":"e_1_2_6_45_1","volume-title":"Neuroscience","author":"Purves D","year":"2001"},{"key":"e_1_2_6_46_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.143.1.171"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200202037"},{"key":"e_1_2_6_48_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200308076"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305673"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(93)90078-Y"},{"key":"e_1_2_6_51_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.901750104"},{"key":"e_1_2_6_52_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.80.19.6126"},{"key":"e_1_2_6_53_1","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/0165-3806(88)90099-5","article-title":"Postnatal growth of medial gastrocnemius motoneurons in the kitten","volume":"471","author":"Tatton WG","year":"1988","journal-title":"Brain Res"},{"key":"e_1_2_6_54_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004410051013"},{"key":"e_1_2_6_55_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.50.4.439"},{"key":"e_1_2_6_56_1","doi-asserted-by":"publisher","DOI":"10.1086\/302369"},{"key":"e_1_2_6_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spen.2006.06.008"},{"key":"e_1_2_6_58_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.18-06-01953.1998"},{"key":"e_1_2_6_59_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.21308"}],"container-title":["Journal of Comparative Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fcne.23010","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cne.23010","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T07:22:37Z","timestamp":1700551357000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/cne.23010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,4,2]]},"references-count":58,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2012,6]]}},"alternative-id":["10.1002\/cne.23010"],"URL":"https:\/\/doi.org\/10.1002\/cne.23010","archive":["Portico"],"relation":{},"ISSN":["0021-9967","1096-9861"],"issn-type":[{"value":"0021-9967","type":"print"},{"value":"1096-9861","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,4,2]]}}}