{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T06:48:39Z","timestamp":1784875719240,"version":"3.55.0"},"reference-count":66,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T00:00:00Z","timestamp":1740096000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["GGP14147"],"award-info":[{"award-number":["GGP14147"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["GGP19099"],"award-info":[{"award-number":["GGP19099"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002721","name":"Charcot\u2013Marie\u2013Tooth Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002721","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fondazione Fronzaroli"},{"name":"Waisman Center from the Eunice Kennedy Shriver National Institute of Child Health and Human Development","award":["P50 HD105353"],"award-info":[{"award-number":["P50 HD105353"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AG046495"],"award-info":[{"award-number":["AG046495"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R21 NS127432"],"award-info":[{"award-number":["R21 NS127432"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,6,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Mutations in myelin protein zero (MPZ) are generally associated with Charcot\u2013Marie\u2013Tooth type 1B (CMT1B) disease, one of the most common forms of demyelinating neuropathy. Pathogenesis of some MPZ mutants, such as S63del and R98C, involves the misfolding and retention of MPZ in the endoplasmic reticulum (ER) of myelinating Schwann cells. To cope with proteotoxic ER stress, Schwann cells mount an unfolded protein response (UPR) characterized by activation of the PERK, ATF6 and IRE1\u03b1\/XBP1 pathways. Previous results showed that targeting the PERK UPR pathway mitigates neuropathy in mouse models of CMT1B; however, the contributions of other UPR pathways in disease pathogenesis remain poorly understood.<\/jats:p>\n                  <jats:p>Here, we probe the importance of IRE1\u03b1\/XBP1 signalling during normal myelination and in CMT1B. In response to ER stress, IRE1\u03b1 is activated to stimulate the non-canonical splicing of Xbp1 mRNA to generate spliced Xbp1 (Xbp1s). This results in the increased expression of the adaptive transcription factor XBP1s, which regulates the expression of genes involved in diverse pathways, including ER proteostasis. We generated mouse models in which Xbp1 is deleted specifically in Schwann cells, preventing XBP1s activation in these cells. We observed that Xbp1 is dispensable for normal developmental myelination, myelin maintenance and remyelination after injury. However, Xbp1 deletion dramatically worsens the hypomyelination and the electrophysiological and locomotor parameters observed in young and adult CMT1B neuropathic animals. RNA-sequencing analysis suggested that XBP1s exerts its adaptive function in CMT1B mouse models in large part via the induction of ER proteostasis genes. Accordingly, the exacerbation of the neuropathy in Xbp1-deficient mice was accompanied by upregulation of ER-stress pathways and of regulated IRE1\u03b1-dependent mRNA decay signalling in Schwann cells, suggesting that the activation of XBP1s via IRE1\u03b1 plays a crucial role in limiting mutant protein toxicity and that this toxicity cannot be compensated by other stress responses. Schwann cell-specific overexpression of XBP1s partly re-established Schwann cell proteostasis and attenuated CMT1B severity in both the S63del and R98C mouse models. In addition, the selective, pharmacological activation of IRE1\u03b1\/XBP1 signalling ameliorated myelination in S63del dorsal root ganglia explants.<\/jats:p>\n                  <jats:p>Collectively, these data show that XBP1 has an essential adaptive role in different models of proteotoxic CMT1B neuropathy and suggest that activation of the IRE1\u03b1\/XBP1 pathway might represent a therapeutic avenue in CMT1B and, possibly, for other neuropathies characterized by UPR activation.<\/jats:p>","DOI":"10.1093\/brain\/awae407","type":"journal-article","created":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T15:27:56Z","timestamp":1734708476000},"page":"1978-1993","source":"Crossref","is-referenced-by-count":9,"title":["Activation of XBP1s attenuates disease severity in models of proteotoxic Charcot\u2013Marie\u2013Tooth type 1B"],"prefix":"10.1093","volume":"148","author":[{"given":"Thierry","family":"Touvier","sequence":"first","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca A","family":"Veneri","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anke","family":"Claessens","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cinzia","family":"Ferri","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Mastrangelo","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"No\u00e9mie","family":"Sorgiati","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Bianchi","sequence":"additional","affiliation":[{"name":"Institute of Experimental Neurology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serena","family":"Valenzano","sequence":"additional","affiliation":[{"name":"Institute of Experimental Neurology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]},{"name":"Department of Biomedical and Clinical Sciences \u2018L. Sacco\u2019, University of Milan , 20157 Milan ,","place":["Italy"]},{"name":"University of Camerino, Center for Neuroscience , 62032 Camerino ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ubaldo","family":"Del Carro","sequence":"additional","affiliation":[{"name":"Institute of Experimental Neurology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Rivellini","sequence":"additional","affiliation":[{"name":"Division of Neuroscience, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Phu","family":"Duong","sequence":"additional","affiliation":[{"name":"Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison , Madison, WI 53706 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael E","family":"Shy","sequence":"additional","affiliation":[{"name":"Department of Neurology, Carver College of Medicine, University of Iowa , Iowa City, IA 52242 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffery W","family":"Kelly","sequence":"additional","affiliation":[{"name":"Department of Chemistry, The Scripps Research Institute , La Jolla, CA 92037 ,","place":["USA"]},{"name":"The Skaggs Institute for Chemical Biology, The Scripps Research Institute , La Jolla, CA 92037 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2963-7921","authenticated-orcid":false,"given":"John","family":"Svaren","sequence":"additional","affiliation":[{"name":"Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison , Madison, WI 53706 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R Luke","family":"Wiseman","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology, The Scripps Research Institute , La Jolla, CA 92037 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6687-9183","authenticated-orcid":false,"given":"Maurizio","family":"D\u2019Antonio","sequence":"additional","affiliation":[{"name":"Division of Genetics and Cell Biology, IRCCS Ospedale San Raffaele , 20132 Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,2,21]]},"reference":[{"key":"2025060300212669100_awae407-B1","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1146\/annurev-neuro-110920-030610","article-title":"Beyond wrapping: Canonical and noncanonical functions of Schwann cells","volume":"45","author":"Taveggia","year":"2022","journal-title":"Annu Rev 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