{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T15:44:03Z","timestamp":1767973443650,"version":"3.49.0"},"reference-count":38,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,12,26]],"date-time":"2013-12-26T00:00:00Z","timestamp":1388016000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Vesicle\u2010associated membrane\u2010protein\u2010associated protein <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content>) is an endoplasmic reticulum (<jats:styled-content style=\"fixed-case\">ER<\/jats:styled-content>) resident protein participating in <jats:styled-content style=\"fixed-case\">ER<\/jats:styled-content> function, vesicle trafficking, calcium homeostasis and lipid transport. Its <jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>\u2010terminal domain, named <jats:styled-content style=\"fixed-case\">MSP<\/jats:styled-content>, is cleaved and secreted, serving as an extracellular ligand. <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> mutations are linked to autosomal\u2010dominant motor neuron diseases, including amyotrophic lateral sclerosis (<jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>) type 8. An altered <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> function is also suspected in sporadic <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The expression pattern of <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> cleavage and secreted products in the peripheral blood leukocytes (<jats:styled-content style=\"fixed-case\">PBL<\/jats:styled-content>) and cerebrospinal fluid (<jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>) of <jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content> patients and neurological controls was assessed. <jats:styled-content style=\"fixed-case\">PBL<\/jats:styled-content> from healthy controls were also analyzed. Assays were carried out through western blotting, using an anti\u2010<jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>\u2010terminal) antibody.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> fragments containing the <jats:styled-content style=\"fixed-case\">MSP<\/jats:styled-content> domain (17\u00a0kDa and 14\u00a0kDa molecular sizes) were identified in <jats:styled-content style=\"fixed-case\">PBL<\/jats:styled-content> of <jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content> and controls, with no significant differences amongst groups. In CSF, only the 14\u00a0kDa <jats:styled-content style=\"fixed-case\">VAPB MSP<\/jats:styled-content> fragment was expressed and a corresponding <jats:styled-content style=\"fixed-case\">VAPA<\/jats:styled-content> fragment was not detected. The <jats:styled-content style=\"fixed-case\">CSF VAPB<\/jats:styled-content> fragment was absent in 58.7% of <jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content> patients, of whom 79.2% were bulbar onset (<jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic>\u00a0=\u00a00.001, bulbar versus spinal).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The absence of the <jats:styled-content style=\"fixed-case\">CSF VAPB MSP<\/jats:styled-content> fragment from most bulbar\u2010onset <jats:styled-content style=\"fixed-case\">SALS<\/jats:styled-content> patients suggests a specific alteration of brain\u2010derived <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> cleavage and secretion in this group of patients, and hints at a role of <jats:styled-content style=\"fixed-case\">VAPB<\/jats:styled-content> in the pathophysiology of this motor neuron disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.12334","type":"journal-article","created":{"date-parts":[[2013,12,26]],"date-time":"2013-12-26T12:00:59Z","timestamp":1388059259000},"page":"478-485","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Expression of vesicle\u2010associated membrane\u2010protein\u2010associated protein <scp>B<\/scp> cleavage products in peripheral blood leukocytes and cerebrospinal fluid of patients with sporadic amyotrophic lateral sclerosis"],"prefix":"10.1111","volume":"21","author":[{"given":"I.","family":"Deidda","sequence":"first","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Galizzi","sequence":"additional","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Passantino","sequence":"additional","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Cascio","sequence":"additional","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Russo","sequence":"additional","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Colletti","sequence":"additional","affiliation":[{"name":"ALS Clinical Research Center Department of Experimental Biomedicine and Clinical Neurosciences University of Palermo Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"La Bella","sequence":"additional","affiliation":[{"name":"ALS Clinical Research Center Department of Experimental Biomedicine and Clinical Neurosciences University of Palermo Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Guarneri","sequence":"additional","affiliation":[{"name":"Neuroscience Unit CNR Institute of Biomedicine and Molecular Immunology Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,12,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.27.070203.144244"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)61156-7"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2009.01290.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181b6bbbd"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0013346"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e328356d328"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneurol.2011.150"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr365"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1086\/425287"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20657"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.04.039"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.161398"},{"key":"e_1_2_8_14_1","first-page":"2950","article-title":"VAPB and C9orf72 mutations in 1 familial amyotrophic lateral sclerosis patient","volume":"33","author":"Blitterswijk M","year":"2012","journal-title":"Neurobiol Aging"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2661-07.2007"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2008.07.005"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2008.03.006"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr559"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2008.05857.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.097345"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.102137"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn040"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2011.07201.x"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2011.12.009"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1080\/146608200300079536"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr351"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410220308"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V11.5.436.436"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1998.9876"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pep.2007.04.007"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2010.03.055"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2009.02815.x"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-303768"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-6146-2"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00702-009-0354-2"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2011.11.026"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00259-011-1979-6"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.05.028"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1326-8-1"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fene.12334","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.12334","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T07:05:20Z","timestamp":1695539120000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.12334"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,12,26]]},"references-count":38,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,3]]}},"alternative-id":["10.1111\/ene.12334"],"URL":"https:\/\/doi.org\/10.1111\/ene.12334","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,12,26]]},"assertion":[{"value":"2013-06-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-11-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-12-26","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}