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| http://purl.uniprot.org/citations/36928510 | http://www.w3.org/2000/01/rdf-schema#comment | "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."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pone.0280650"xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/author | "Can M."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/author | "Seyrantepe V."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/author | "Ates N."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/author | "Sengul T."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/date | "2023"xsd:gYear |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/name | "PLoS One"xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/pages | "e0280650"xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/title | "Autophagic flux is impaired in the brain tissue of Tay-Sachs disease mouse model."xsd:string |
| http://purl.uniprot.org/citations/36928510 | http://purl.uniprot.org/core/volume | "18"xsd:string |
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