{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T04:47:05Z","timestamp":1791780425586,"version":"4.3.4"},"reference-count":56,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS111715"],"award-info":[{"award-number":["R01NS111715"]}],"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":["R01NS112327"],"award-info":[{"award-number":["R01NS112327"]}],"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":["F31NS124106"],"award-info":[{"award-number":["F31NS124106"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100014177","name":"Rosenau Family Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100014177","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Glia"],"published-print":{"date-parts":[[2026,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Krabbe disease (KD) is a lysosomal storage disorder characterized by severe peripheral demyelination caused by mutations in galactosylceramidase (GALC), leading to the accumulation of galactosylceramide and psychosine. Although psychosine toxicity is central to KD pathology, additional mechanisms likely contribute to myelin dysfunction. Because Schwann cells (SCs) play a critical role in myelin maintenance, and GALC deficiency disrupts lysosomal function, we hypothesized that autophagy is altered in SCs during KD progression. Using a SC\u2010specific\n                    <jats:italic>Galc<\/jats:italic>\n                    conditional knockout (\n                    <jats:italic>Galc<\/jats:italic>\n                    SC cKO) mouse model, we identified early changes in autophagy\u2010related protein levels and increased autophagosome abundance during the pre\u2010symptomatic phase, prior to detectable myelin abnormalities. To assess whether autophagy contributes to disease progression, we selectively deleted\n                    <jats:italic>Atg7<\/jats:italic>\n                    , an essential regulator of canonical autophagy, in SCs.\n                    <jats:italic>Atg7<\/jats:italic>\n                    deletion alone did not affect myelin or behavior, but its loss in\n                    <jats:italic>Galc<\/jats:italic>\n                    SC cKO mice markedly worsened disease severity.\n                    <jats:italic>Atg7\u2010Galc<\/jats:italic>\n                    SC cKO mice exhibited significantly thinner myelin and pronounced deficits in motor performance and nerve conduction, accompanied by altered steady\u2010state levels of autophagy\u2010associated proteins and functional decline. Notably,\n                    <jats:italic>Atg7<\/jats:italic>\n                    deletion in\n                    <jats:italic>Galc<\/jats:italic>\n                    SC cKO mice did not further increase macrophage abundance, suggesting that the worsened myelin phenotype occurs without additional macrophage recruitment at this stage. Together, these findings demonstrate that autophagy\u2010associated changes occur early in KD and ATG7\u2010dependent pathways play a protective role during disease progression. Modulating ATG7\u2010dependent pathways may therefore warrant further investigation as a strategy to support myelin maintenance in KD and related lysosomal disorders.\n                  <\/jats:p>","DOI":"10.1002\/glia.70244","type":"journal-article","created":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T04:26:09Z","timestamp":1791779169000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Schwann Cell\u2010Specific\n                    <scp>ATG7<\/scp>\n                    \u2010Dependent Autophagy Protects Myelin in a Mouse Model of Krabbe Disease"],"prefix":"10.1002","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1056-0672","authenticated-orcid":false,"given":"Narayan","family":"Dhimal","sequence":"first","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Neuroscience Program Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-5009-9290","authenticated-orcid":false,"given":"Aditya","family":"Sidharth","sequence":"additional","affiliation":[{"name":"Neuroscience Program Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Department of Biotechnical and Clinical Laboratory Sciences Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiajun","family":"Zhang","sequence":"additional","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Trina","family":"Rudra","sequence":"additional","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1967-8933","authenticated-orcid":false,"given":"Edward","family":"Hurley","sequence":"additional","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9362-9454","authenticated-orcid":false,"given":"Pablo M.","family":"Paez","sequence":"additional","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Neuroscience Program Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Department of Pharmacology and Toxicology Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3942-6637","authenticated-orcid":false,"given":"Fraser J.","family":"Sim","sequence":"additional","affiliation":[{"name":"Neuroscience Program Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Department of Pharmacology and Toxicology Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5285-3820","authenticated-orcid":false,"given":"Daesung","family":"Shin","sequence":"additional","affiliation":[{"name":"Institute of Myelin and Glia Exploration Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Neuroscience Program Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"},{"name":"Department of Biotechnical and Clinical Laboratory Sciences Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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