{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T22:09:17Z","timestamp":1790201357362,"version":"4.1.0"},"reference-count":48,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2025,1,5]],"date-time":"2025-01-05T00:00:00Z","timestamp":1736035200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health","award":["GSA23C003"],"award-info":[{"award-number":["GSA23C003"]}]},{"name":"Italian Ministry of Health","award":["GGP20011"],"award-info":[{"award-number":["GGP20011"]}]},{"DOI":"10.13039\/501100002426","name":"Telethon Foundation","doi-asserted-by":"publisher","award":["GSA23C003"],"award-info":[{"award-number":["GSA23C003"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002426","name":"Telethon Foundation","doi-asserted-by":"publisher","award":["GGP20011"],"award-info":[{"award-number":["GGP20011"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>CLN8 and other neuronal ceroid lipofuscinoses (NCLs) often lead to cognitive decline, emotional disturbances, and social deficits, worsening with disease progression. Disrupted lysosomal pH, impaired autophagy, and defective dendritic arborization contribute to these symptoms. Using a cln8\u2212\/\u2212 zebrafish model, we identified significant impairments in locomotion, anxiety, and aggression, along with subtle deficits in social interactions, positioning zebrafish as a useful model for therapeutic studies in NCL. Our findings show that trehalose, an autophagy enhancer, ameliorates anxiety, and modestly improves social behavior and predator avoidance in mutant zebrafish. This finding aligns animal models with clinical reports suggestive of behavioral improvements in NCL patients. Trehalose holds promise as a therapeutic agent for CLN8, warranting further research into its neuroprotective mechanisms and clinical applications.<\/jats:p>","DOI":"10.3390\/cells14010055","type":"journal-article","created":{"date-parts":[[2025,1,6]],"date-time":"2025-01-06T09:17:02Z","timestamp":1736155022000},"page":"55","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Trehalose Ameliorates Zebrafish Emotional and Social Deficits Caused by CLN8 Dysfunction"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4879-6610","authenticated-orcid":false,"given":"Rosario","family":"Licitra","sequence":"first","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"},{"name":"Department of Veterinary Sciences, University of Pisa, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Della Vecchia","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Santucci","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachele","family":"Vivarelli","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Bernardi","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo M.","family":"Santorelli","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0194-0702","authenticated-orcid":false,"given":"Maria","family":"Marchese","sequence":"additional","affiliation":[{"name":"Neurobiology and Molecular Medicine Unit, IRCCS Fondazione Stella Maris, 56128 Calambrone, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1684\/epd.2016.0844","article-title":"Neuronal ceroid lipofuscinoses","volume":"18","author":"Nita","year":"2016","journal-title":"Epileptic Disord."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1370","DOI":"10.1038\/s41556-018-0228-7","article-title":"CLN8 is an endoplasmic reticulum cargo receptor that regulates lysosome biogenesis","volume":"20","author":"Bajaj","year":"2018","journal-title":"Nat. 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