Autophagic Flux Is Impaired in the Brain Tissue of Tay-Sachs Disease Mouse Model
| dc.contributor.author | Şengül, Tuğçe | |
| dc.contributor.author | Can, Melike | |
| dc.contributor.author | Ateş, Nurselin | |
| dc.contributor.author | Seyrantepe, Volkan | |
| dc.date.accessioned | 2023-04-19T12:39:44Z | |
| dc.date.available | 2023-04-19T12:39:44Z | |
| dc.date.issued | 2023 | |
| dc.description.abstract | Tay-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.sponsorship | Funding: This study is funded by TUBİTAK Grant No:215Z083 | en_US |
| dc.identifier.doi | 10.1371/journal.pone.0280650 | |
| dc.identifier.issn | 1932-6203 | |
| dc.identifier.scopus | 2-s2.0-85150230228 | |
| dc.identifier.uri | https://doi.org/10.1371/journal.pone.0280650 | |
| dc.identifier.uri | https://hdl.handle.net/11147/13389 | |
| dc.language.iso | en | en_US |
| dc.publisher | Public Library of Science | en_US |
| dc.relation | Erken Başlangıçlı Tay-Sachs Hastalığı Fare Modelinde Hücresel Patolojinin Araştırılması | tr |
| dc.relation.ispartof | PLoS ONE | en_US |
| dc.rights | info:eu-repo/semantics/openAccess | en_US |
| dc.subject | Beta-N-Acetylhexosaminidases | en_US |
| dc.subject | Gangliosides | en_US |
| dc.subject | Animals | en_US |
| dc.subject | Autophagy | en_US |
| dc.subject | Brain | en_US |
| dc.subject | Tay-Sachs disease | en_US |
| dc.title | Autophagic Flux Is Impaired in the Brain Tissue of Tay-Sachs Disease Mouse Model | en_US |
| dc.type | Article | en_US |
| dspace.entity.type | Publication | |
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| gdc.coar.access | open access | |
| gdc.coar.type | text::journal::journal article | |
| gdc.collaboration.industrial | false | |
| gdc.description.department | İzmir Institute of Technology. Molecular Biology and Genetics | en_US |
| gdc.description.issue | 3 March | en_US |
| gdc.description.publicationcategory | Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı | en_US |
| gdc.description.scopusquality | Q1 | |
| gdc.description.volume | 18 | en_US |
| gdc.description.wosquality | Q2 | |
| gdc.identifier.openalex | W4327565218 | |
| gdc.identifier.pmid | 36928510 | |
| gdc.identifier.wos | WOS:000985134400073 | |
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| gdc.oaire.keywords | Tay-Sachs Disease | |
| gdc.oaire.keywords | Science | |
| gdc.oaire.keywords | Q | |
| gdc.oaire.keywords | R | |
| gdc.oaire.keywords | Brain | |
| gdc.oaire.keywords | G(M2) Ganglioside | |
| gdc.oaire.keywords | beta-N-Acetylhexosaminidases | |
| gdc.oaire.keywords | Mice | |
| gdc.oaire.keywords | Disease Models, Animal | |
| gdc.oaire.keywords | Hexosaminidase A | |
| gdc.oaire.keywords | Autophagy | |
| gdc.oaire.keywords | Medicine | |
| gdc.oaire.keywords | Animals | |
| gdc.oaire.keywords | Research Article | |
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