{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T11:47:35Z","timestamp":1791978455172,"version":"4.4.0"},"reference-count":55,"publisher":"Rockefeller University Press","issue":"1","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2005,7,4]]},"abstract":"<jats:p>Lysosomal storage is the most common cause of neurodegenerative brain disease in preadulthood. However, the underlying cellular mechanisms that lead to neuronal dysfunction are unknown. Here, we report that loss of Drosophila benchwarmer (bnch), a predicted lysosomal sugar carrier, leads to carbohydrate storage in yolk spheres during oogenesis and results in widespread accumulation of enlarged lysosomal and late endosomal inclusions. At the bnch larval neuromuscular junction, we observe similar inclusions and find defects in synaptic vesicle recycling at the level of endocytosis. In addition, loss of bnch slows endosome-to-lysosome trafficking in larval garland cells. In adultbnch flies, we observe age-dependent synaptic dysfunction and neuronal degeneration. Finally, we find that loss of bnch strongly enhances tau neurotoxicity in a dose-dependent manner. We hypothesize that, in bnch, defective lysosomal carbohydrate efflux leads to endocytic defects with functional consequences in synaptic strength, neuronal viability, and tau neurotoxicity.<\/jats:p>","DOI":"10.1083\/jcb.200412001","type":"journal-article","created":{"date-parts":[[2005,7,5]],"date-time":"2005-07-05T18:28:32Z","timestamp":1120588112000},"page":"127-139","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":130,"title":["Aberrant lysosomal carbohydrate storage accompanies endocytic defects and neurodegeneration in<i>Drosophila benchwarmer<\/i>"],"prefix":"10.1083","volume":"170","author":[{"given":"Bart","family":"Dermaut","sequence":"first","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koenraad K.","family":"Norga","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"},{"name":"2Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX 77030"},{"name":"4Laboratory of Developmental Genetics, Flanders Interuniversity Institute of Biotechnology (VIB), University of Leuven, B-3000 Leuven, Belgium"},{"name":"5Division of Pediatrics, University of Leuven, School of Medicine, B-3000 Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Artur","family":"Kania","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrik","family":"Verstreken","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongling","family":"Pan","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Zhou","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Callaerts","sequence":"additional","affiliation":[{"name":"4Laboratory of Developmental Genetics, Flanders Interuniversity Institute of Biotechnology (VIB), University of Leuven, B-3000 Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo J.","family":"Bellen","sequence":"additional","affiliation":[{"name":"1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030"},{"name":"2Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX 77030"},{"name":"3Program in Developmental Biology, Baylor College of Medicine, Houston, TX 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2005,7,5]]},"reference":[{"key":"2023072906591998300_BIB1","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1126\/science.172.3987.1055","volume":"172","year":"1971","journal-title":"Science."},{"key":"2023072906591998300_BIB2","doi-asserted-by":"crossref","first-page":"2270","DOI":"10.1126\/science.1072152","volume":"297","year":"2002","journal-title":"Science."},{"key":"2023072906591998300_BIB3","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/BF00318566","volume":"90","year":"1995","journal-title":"Acta Neuropathol. 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