{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T17:50:05Z","timestamp":1790272205079,"version":"4.1.0"},"reference-count":36,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T00:00:00Z","timestamp":1678924800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004410","name":"TUBITAK","doi-asserted-by":"crossref","award":["215Z083"],"award-info":[{"award-number":["215Z083"]}],"id":[{"id":"10.13039\/501100004410","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>Tay-Sachs disease is a lethal lysosomal storage disorder caused by mutations in the HexA gene encoding the \u03b1 subunit of the lysosomal \u03b2-hexosaminidase enzyme (HEXA). Abnormal GM2 ganglioside accumulation causes progressive deterioration in the central nervous system in Tay-Sachs patients. <jats:italic>Hexa-\/-<\/jats:italic> mouse model failed to display abnormal phenotype. Recently, our group generated <jats:italic>Hexa-\/-Neu3-\/-<\/jats:italic> mouse showed severe neuropathological indications similar to Tay-Sachs patients. Despite excessive GM2 ganglioside accumulation in the brain and visceral organs, the regulation of autophagy has not been clarified yet in the Tay-Sachs disease mouse model. Therefore, we investigated distinct steps of autophagic flux using markers including LC3 and p62 in four different brain regions from the <jats:italic>Hexa-\/-Neu3-\/-<\/jats:italic> mice model of Tay-Sachs disease. Our data revealed accumulated autophagosomes and autophagolysosomes indicating impairment in autophagic flux in the brain. We suggest that autophagy might be a new therapeutic target for the treatment of devastating Tay-Sachs disease.<\/jats:p>","DOI":"10.1371\/journal.pone.0280650","type":"journal-article","created":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T17:49:53Z","timestamp":1678988993000},"page":"e0280650","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":9,"title":["Autophagic flux is impaired in the brain tissue of Tay-Sachs disease mouse model"],"prefix":"10.1371","volume":"18","author":[{"given":"Tugce","family":"Sengul","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melike","family":"Can","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nurselin","family":"Ate\u015f","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0243-5011","authenticated-orcid":true,"given":"Volkan","family":"Seyrantepe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2023,3,16]]},"reference":[{"key":"pone.0280650.ref001","first-page":"3827","article-title":"The GM2 Gangliosidoses. 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