| |  Online-Ressource |
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| Verfasst von: | Peters, Judith [VerfasserIn]  |
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| | Rittger, Andrea [VerfasserIn] |
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| | Weisner, Rebecca [VerfasserIn] |
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| | Knabbe, Johannes [VerfasserIn] |
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| | Zunke, Friederike [VerfasserIn] |
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| | Rothaug, Michelle [VerfasserIn] |
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| | Damme, Markus [VerfasserIn] |
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| | Berkovic, Samuel F. [VerfasserIn] |
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| | Blanz, Judith [VerfasserIn] |
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| | Saftig, Paul [VerfasserIn] |
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| | Schwake, Michael [VerfasserIn] |
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| Titel: | Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease |
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| Verf.angabe: | Judith Peters, Andrea Rittger, Rebecca Weisner, Johannes Knabbe, Friederike Zunke, Michelle Rothaug, Markus Damme, Samuel F. Berkovic, Judith Blanz, Paul Saftig, Michael Schwake |
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| E-Jahr: | 2015 |
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| Jahr: | 7 January 2015 |
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| Umfang: | 7 S. |
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| Fussnoten: | Gesehen am 11.02.2021 |
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| Titel Quelle: | Enthalten in: Biochemical and biophysical research communications |
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| Ort Quelle: | Orlando, Fla. : Academic Press, 1959 |
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| Jahr Quelle: | 2015 |
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| Band/Heft Quelle: | 457(2015), 3, Seite 334-340 |
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| ISSN Quelle: | 1090-2104 |
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| Abstract: | The lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) has been identified as a receptor for enterovirus 71 uptake and mannose-6-phosphate-independent lysosomal trafficking of the acid hydrolase β-glucocerebrosidase. Here we show that LIMP-2 undergoes proteolytic cleavage mediated by lysosomal cysteine proteases. Heterologous expression and in vitro studies suggest that cathepsin-F is mainly responsible for the lysosomal processing of wild-type LIMP-2. Furthermore, examination of purified lysosomes revealed that LIMP-2 undergoes proteolysis in vivo. Mutations in the gene encoding cathepsin-F (CTSF) have recently been associated with type-B-Kufs-disease, an adult form of neuronal ceroid-lipofuscinosis. In this study we show that disease-causing cathepsin-F mutants fail to cleave LIMP-2. Our findings provide evidence that LIMP-2 represents an in vivo substrate of cathepsin-F with relevance for understanding the pathophysiology of type-B-Kufs-disease. |
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| DOI: | doi:10.1016/j.bbrc.2014.12.111 |
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| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
Volltext: https://doi.org/10.1016/j.bbrc.2014.12.111 |
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| | Volltext: https://www.sciencedirect.com/science/article/pii/S0006291X14023250 |
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| | DOI: https://doi.org/10.1016/j.bbrc.2014.12.111 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| Sach-SW: | Cathepsin-F |
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| | Kufs disease |
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| | LIMP-2 |
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| | Lysosomal storage disease |
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| | Neuronal ceroid-lipofuscinosis |
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| K10plus-PPN: | 1748105272 |
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| Verknüpfungen: | → Zeitschrift |
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Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease / Peters, Judith [VerfasserIn]; 7 January 2015 (Online-Ressource)
