Please use this identifier to cite or link to this item: https://hdl.handle.net/11147/13389
Title: Autophagic flux is impaired in the brain tissue of Tay-Sachs disease mouse model
Authors: Şengül, Tuğçe
Can, Melike
Ateş, Nurselin
Seyrantepe, Volkan
Keywords: Beta-N-Acetylhexosaminidases
Gangliosides
Animals
Autophagy
Brain
Tay-Sachs disease
Issue Date: 2023
Publisher: Public Library of Science
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.
URI: https://doi.org/10.1371/journal.pone.0280650
https://hdl.handle.net/11147/13389
ISSN: 1932-6203
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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