pH-dependent localization of Btn1p in the yeast model for Batten disease
SUMMARY 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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
The Company of Biologists
2011-01-01
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| Series: | Disease Models & Mechanisms |
| Online Access: | http://dmm.biologists.org/content/4/1/120 |
| _version_ | 1811271869014212608 |
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| author | Devin M. Wolfe Sergio Padilla-Lopez Seasson Phillips Vitiello David A. Pearce |
| author_facet | Devin M. Wolfe Sergio Padilla-Lopez Seasson Phillips Vitiello David A. Pearce |
| author_sort | Devin M. Wolfe |
| collection | DOAJ |
| description | SUMMARY
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. |
| first_indexed | 2024-04-12T22:29:55Z |
| format | Article |
| id | doaj.art-c39f5d05dd954ae490c55909f8396b51 |
| institution | Directory Open Access Journal |
| issn | 1754-8403 1754-8411 |
| language | English |
| last_indexed | 2024-04-12T22:29:55Z |
| publishDate | 2011-01-01 |
| publisher | The Company of Biologists |
| record_format | Article |
| series | Disease Models & Mechanisms |
| spelling | doaj.art-c39f5d05dd954ae490c55909f8396b512022-12-22T03:14:01ZengThe Company of BiologistsDisease Models & Mechanisms1754-84031754-84112011-01-014112012510.1242/dmm.006114006114pH-dependent localization of Btn1p in the yeast model for Batten diseaseDevin M. WolfeSergio Padilla-LopezSeasson Phillips VitielloDavid A. PearceSUMMARY 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.http://dmm.biologists.org/content/4/1/120 |
| spellingShingle | Devin M. Wolfe Sergio Padilla-Lopez Seasson Phillips Vitiello David A. Pearce pH-dependent localization of Btn1p in the yeast model for Batten disease Disease Models & Mechanisms |
| title | pH-dependent localization of Btn1p in the yeast model for Batten disease |
| title_full | pH-dependent localization of Btn1p in the yeast model for Batten disease |
| title_fullStr | pH-dependent localization of Btn1p in the yeast model for Batten disease |
| title_full_unstemmed | pH-dependent localization of Btn1p in the yeast model for Batten disease |
| title_short | pH-dependent localization of Btn1p in the yeast model for Batten disease |
| title_sort | ph dependent localization of btn1p in the yeast model for batten disease |
| url | http://dmm.biologists.org/content/4/1/120 |
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