{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:24:33Z","timestamp":1790832273554,"version":"4.1.0"},"reference-count":167,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2021,7,31]],"date-time":"2021-07-31T00:00:00Z","timestamp":1627689600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002261","name":"Russian Foundation for Basic Research","doi-asserted-by":"publisher","award":["20-34-90148"],"award-info":[{"award-number":["20-34-90148"]}],"id":[{"id":"10.13039\/501100002261","id-type":"DOI","asserted-by":"publisher"}]},{"name":"IPAC RAS State Targets Project","award":["0090-2019-0005"],"award-info":[{"award-number":["0090-2019-0005"]}]},{"name":"Volkswagen Stiftung","award":["90218 and 97750"],"award-info":[{"award-number":["90218 and 97750"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Various neurodegenerative disorders are associated with human NTE\/PNPLA6 dysfunction. Mechanisms of neuropathogenesis in these diseases are far from clearly elucidated. Hereditary spastic paraplegia belongs to a type of neurodegeneration associated with NTE\/PNLPLA6 and is implicated in neuron death. In this study, we used Drosophila melanogaster to investigate the consequences of neuronal knockdown of swiss cheese (sws)\u2014the evolutionarily conserved ortholog of human NTE\/PNPLA6\u2014in vivo. Adult flies with the knockdown show longevity decline, locomotor and memory deficits, severe neurodegeneration progression in the brain, reactive oxygen species level acceleration, mitochondria abnormalities and lipid droplet accumulation. Our results suggest that SWS\/NTE\/PNPLA6 dysfunction in neurons induces oxidative stress and lipid metabolism alterations, involving mitochondria dynamics and lipid droplet turnover in neurodegeneration pathogenesis. We propose that there is a complex mechanism in neurological diseases such as hereditary spastic paraplegia, which includes a stress reaction, engaging mitochondria, lipid droplets and endoplasmic reticulum interplay.<\/jats:p>","DOI":"10.3390\/ijms22158275","type":"journal-article","created":{"date-parts":[[2021,8,1]],"date-time":"2021-08-01T21:51:07Z","timestamp":1627854667000},"page":"8275","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Loss of swiss cheese in Neurons Contributes to Neurodegeneration with Mitochondria Abnormalities, Reactive Oxygen Species Acceleration and Accumulation of Lipid Droplets in Drosophila Brain"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0620-4747","authenticated-orcid":false,"given":"Pavel A.","family":"Melentev","sequence":"first","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0813-6722","authenticated-orcid":false,"given":"Elena V.","family":"Ryabova","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7863-1748","authenticated-orcid":false,"given":"Nina V.","family":"Surina","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darya R.","family":"Zhmujdina","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3564-1698","authenticated-orcid":false,"given":"Artem E.","family":"Komissarov","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9576-9482","authenticated-orcid":false,"given":"Ekaterina A.","family":"Ivanova","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7171-8832","authenticated-orcid":false,"given":"Natalia P.","family":"Boltneva","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds Russian Academy of Sciences, 142432 Chernogolovka, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8136-6906","authenticated-orcid":false,"given":"Galina F.","family":"Makhaeva","sequence":"additional","affiliation":[{"name":"Institute of Physiologically Active Compounds Russian Academy of Sciences, 142432 Chernogolovka, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana I.","family":"Sliusarenko","sequence":"additional","affiliation":[{"name":"Institute of Cell Biochemistry, Hannover Medical School, 30625 Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andriy S.","family":"Yatsenko","sequence":"additional","affiliation":[{"name":"Institute of Cell Biochemistry, Hannover Medical School, 30625 Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iryna I.","family":"Mohylyak","sequence":"additional","affiliation":[{"name":"Department of Genetics and Biotechnology, Ivan Franko National University of Lviv, 79005 Lviv, Ukraine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1582-2037","authenticated-orcid":false,"given":"Nataliya P.","family":"Matiytsiv","sequence":"additional","affiliation":[{"name":"Department of Genetics and Biotechnology, Ivan Franko National University of Lviv, 79005 Lviv, Ukraine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3855-0345","authenticated-orcid":false,"given":"Halyna R.","family":"Shcherbata","sequence":"additional","affiliation":[{"name":"Institute of Cell Biochemistry, Hannover Medical School, 30625 Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svetlana V.","family":"Sarantseva","sequence":"additional","affiliation":[{"name":"Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of NRC \u00abKurchatov Institute\u00bb, 188300 Gatchina, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,7,31]]},"reference":[{"key":"ref_1","first-page":"1009","article-title":"Is the transportation highway the right road for hereditary spastic paraplegia?","volume":"71","author":"Crosby","year":"2002","journal-title":"Am. J. Med. 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