{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:13:01Z","timestamp":1787015581498,"version":"build-2736575974"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T00:00:00Z","timestamp":1754956800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100002721","name":"Charcot-Marie-Tooth Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002721","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R03 NS123787"],"award-info":[{"award-number":["R03 NS123787"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R37 NS054154"],"award-info":[{"award-number":["R37 NS054154"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000052","name":"NIH Office of the Director","doi-asserted-by":"publisher","award":["U54 OD030187"],"award-info":[{"award-number":["U54 OD030187"]}],"id":[{"id":"10.13039\/100000052","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["CA34196"],"award-info":[{"award-number":["CA34196"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2025,9]]},"abstract":"ABSTRACT<\/jats:title>\n \n Background<\/jats:title>\n Degeneration of peripheral motor and sensory axons is a key aspect of the pathophysiology of Charcot\u2013Marie\u2013Tooth disease and related inherited neurodegenerative conditions.<\/jats:p>\n <\/jats:sec>\n \n Aims<\/jats:title>\n \n Given that mutations in many (>\u2009100) genes can cause these disorders, it is unclear if a generalized therapeutic strategy can be identified that will apply across these disease subtypes; however, strategies to prevent or slow axon degeneration are attractive candidates. Wallerian axon degeneration is an active process following insults such as nerve injury, and SARM1 is a central mediator of this process. When SARM1 is inhibited, axons distal to the site of injury persist for weeks rather than degenerating. In addition, SARM1 inhibition or genetic deletion has been shown to provide benefit in acquired neuropathies such as diabetic\/metabolic neuropathy and chemotherapy\u2010induced neuropathy in animal models. Here we examined the effects of genetically deleting\n Sarm1<\/jats:italic>\n in mouse models of CMT.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n We bred knockout mice lacking\n Sarm1<\/jats:italic>\n to three different mouse models of CMT or related disorders. These include mice lacking\n Gjb1<\/jats:italic>\n , modeling CMT1X, mice with mutations in\n Kif1a<\/jats:italic>\n , modeling hereditary sensory neuropathy IIC and spastic paraplegia type 30, and mice lacking\n Fig4<\/jats:italic>\n , modeling CMT4J and Yunis\u2010Varon syndrome. Clinically relevant outcomes measures including survival (\n Kif1a<\/jats:italic>\n and\n Fig4<\/jats:italic>\n ), grip strength and motor behavior, peripheral neurophysiology, molecular biomarkers, and nerve histopathology were assessed for each model with and without\n Sarm1<\/jats:italic>\n expression.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n No improvement in the mutant phenotype was found for any model, although elevated levels of circulating neurofilament light chain levels were delayed in the\n Fig4<\/jats:italic>\n mice.\n Kif1a<\/jats:italic>\n mice showed deficits slightly earlier in the absence of\n Sarm1<\/jats:italic>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Interpretation<\/jats:title>\n \n While we found no benefit from deleting\n Sarm1<\/jats:italic>\n in these mouse models, they were chosen for their human disease relevance and not for biochemical indicators that SARM1 may be a good target. Thus, SARM1 inhibition may still be effective in other forms of inherited neuropathy, but additional research will be required to identify those candidate subtypes.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/jns.70052","type":"journal-article","created":{"date-parts":[[2025,8,13]],"date-time":"2025-08-13T06:00:14Z","timestamp":1755064814000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Genetic Deletion of\n Sarm1<\/i>\n in Mouse Models of Three Neurological Diseases"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4387-4782","authenticated-orcid":false,"given":"Courtney L.","family":"Hatton","sequence":"first","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Terrey","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maximiliano","family":"Presa","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Ryan","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Perkins","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicki","family":"Kennedy","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cathleen M.","family":"Lutz","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9229-3407","authenticated-orcid":false,"given":"Robert W.","family":"Burgess","sequence":"additional","affiliation":[{"name":"The Jackson Laboratory Bar Harbor Maine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,8,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2014.04.003"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn3680"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1197-13.2013"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1223899"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.1000300"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2017.02.022"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2021.02.009"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311-021-01125-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.129920"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI161566"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12219"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2019.146539"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000735"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12588"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature05876"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-60761-058-8_20"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1809430115"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.16144"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/joa.13605"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12477"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2017.03.009"},{"key":"e_1_2_8_23_1","article-title":"Assessment of Spontaneous Alternation, Novel Object Recognition and Limb Clasping in Transgenic Mouse Models of Amyloid\u2010Beta and tau Neuropathology","volume":"123","author":"Miedel C. 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