Please use this identifier to cite or link to this item: https://hdl.handle.net/11147/13389
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dc.contributor.authorŞengül, Tuğçe-
dc.contributor.authorCan, Melike-
dc.contributor.authorAteş, Nurselin-
dc.contributor.authorSeyrantepe, Volkan-
dc.date.accessioned2023-04-19T12:39:44Z-
dc.date.available2023-04-19T12:39:44Z-
dc.date.issued2023-
dc.identifier.issn1932-6203-
dc.identifier.urihttps://doi.org/10.1371/journal.pone.0280650-
dc.identifier.urihttps://hdl.handle.net/11147/13389-
dc.description.abstractTay-Sachs disease is a lethal lysosomal storage disorder caused by mutations in the HexA gene encoding the α subunit of the lysosomal β-hexosaminidase enzyme (HEXA). Abnormal GM2 ganglioside accumulation causes progressive deterioration in the central nervous system in Tay-Sachs patients. Hexa-/-mouse model failed to display abnormal phenotype. Recently, our group generated Hexa-/-Neu3-/-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 Hexa-/-Neu3-/-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. © 2023 Sengul et al.en_US
dc.description.sponsorshipFunding: This study is funded by TUBİTAK Grant No:215Z083en_US
dc.language.isoenen_US
dc.publisherPublic Library of Scienceen_US
dc.relationErken Başlangıçlı Tay-Sachs Hastalığı Fare Modelinde Hücresel Patolojinin Araştırılmasıtr
dc.relation.ispartofPLoS ONEen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectBeta-N-Acetylhexosaminidasesen_US
dc.subjectGangliosidesen_US
dc.subjectAnimalsen_US
dc.subjectAutophagyen_US
dc.subjectBrainen_US
dc.subjectTay-Sachs diseaseen_US
dc.titleAutophagic flux is impaired in the brain tissue of Tay-Sachs disease mouse modelen_US
dc.typeArticleen_US
dc.departmentİzmir Institute of Technology. Molecular Biology and Geneticsen_US
dc.identifier.volume18en_US
dc.identifier.issue3 Marchen_US
dc.identifier.wosWOS:000985134400073en_US
dc.identifier.scopus2-s2.0-85150230228en_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.identifier.doi10.1371/journal.pone.0280650-
dc.identifier.pmid36928510en_US
dc.relation.grantno215Z083-
dc.authorscopusid57856462600-
dc.authorscopusid57855836700-
dc.authorscopusid57195980858-
dc.authorscopusid6602725956-
dc.identifier.scopusqualityQ1-
item.grantfulltextopen-
item.openairetypeArticle-
item.fulltextWith Fulltext-
item.cerifentitytypePublications-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.languageiso639-1en-
crisitem.author.dept04.03. Department of Molecular Biology and Genetics-
crisitem.author.dept04.03. Department of Molecular Biology and Genetics-
Appears in Collections:Molecular Biology and Genetics / Moleküler Biyoloji ve Genetik
PubMed İndeksli Yayınlar Koleksiyonu / PubMed Indexed Publications Collection
Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection
WoS İndeksli Yayınlar Koleksiyonu / WoS Indexed Publications Collection
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