{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T04:03:23Z","timestamp":1779336203373,"version":"3.51.4"},"reference-count":37,"publisher":"The Company of Biologists","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,1,1]]},"abstract":"<jats:title>SUMMARY<\/jats:title>\n               <jats:p>Btn1p the yeast homolog of human CLN3, which is associated with juvenile Batten disease has been implicated in several cellular pathways. Yeast cells lacking BTN1 are unable to couple ATP hydrolysis and proton pumping activities by the vacuolar ATPase (V-ATPase). In this work, we demonstrate that changes in extracellular pH result in altered transcription of BTN1, as well as a change in the glycosylation state and localization of Btn1p. At high pH, Btn1p expression was increased and the protein was mainly located in vacuolar membranes. However, low pH decreased Btn1p expression and changed its location to undefined punctate membranes. Moreover, our results suggest that differential Btn1p localization may be regulated by its glycosylation state. Underlying pathogenic implications for Batten disease of altered cellular distribution of CLN3 are discussed.<\/jats:p>","DOI":"10.1242\/dmm.006114","type":"journal-article","created":{"date-parts":[[2010,10,20]],"date-time":"2010-10-20T04:13:19Z","timestamp":1287547999000},"page":"120-125","source":"Crossref","is-referenced-by-count":9,"title":["pH-dependent localization of Btn1p in the yeast model for Batten disease"],"prefix":"10.1242","volume":"4","author":[{"given":"Devin M.","family":"Wolfe","sequence":"first","affiliation":[{"name":"Center for Neural Development and Disease, Aab Institute of Biomedical Sciences, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sergio","family":"Padilla-Lopez","sequence":"additional","affiliation":[{"name":"Center for Neural Development and Disease, Aab Institute of Biomedical Sciences, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seasson Phillips","family":"Vitiello","sequence":"additional","affiliation":[{"name":"Center for Neural Development and Disease, Aab Institute of Biomedical Sciences, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Pearce","sequence":"additional","affiliation":[{"name":"Center for Neural Development and Disease, Aab Institute of Biomedical Sciences, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"},{"name":"Department of Biochemistry and Biophysics University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"},{"name":"Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","reference":[{"key":"2021042612465847600_b1-0040120","doi-asserted-by":"crossref","first-page":"3075","DOI":"10.1021\/bi00433a051","article-title":"Interaction of anions and ATP with the coated vesicle proton pump","volume":"28","author":"Arai","year":"1989","journal-title":"Biochemistry"},{"key":"2021042612465847600_b2-0040120","doi-asserted-by":"crossref","first-page":"28872","DOI":"10.1074\/jbc.M300943200","article-title":"Sphingolipid requirement for generation of a functional v1 component of the vacuolar ATPase","volume":"278","author":"Chung","year":"2003","journal-title":"J. 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Sci."},{"key":"2021042612465847600_b9-0040120","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1242\/jeb.203.1.61","article-title":"Composition and assembly of the yeast vacuolar H(+)-ATPase complex","volume":"203","author":"Graham","year":"2000","journal-title":"J. Exp. Biol."},{"key":"2021042612465847600_b10-0040120","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1093\/nar\/24.13.2519","article-title":"A new efficient gene disruption cassette for repeated use in budding yeast","volume":"24","author":"Guldener","year":"1996","journal-title":"Nucleic Acids Res."},{"key":"2021042612465847600_b11-0040120","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1093\/hmg\/7.1.85","article-title":"Biosynthesis and intracellular targeting of the CLN3 protein defective in Batten disease","volume":"7","author":"Jarvela","year":"1998","journal-title":"Hum. Mol. 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