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PrP overdrive

Does inhibition of α-cleavage contribute to PrPC toxicity and prion disease?

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Pages 183-191 | Received 10 Feb 2014, Accepted 05 Apr 2014, Published online: 10 Apr 2014

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Santra Brenna, Hermann C. Altmeppen, Behnam Mohammadi, Björn Rissiek, Florence Schlink, Peter Ludewig, Christoph Krisp, Hartmut Schlüter, Antonio Virgilio Failla, Carola Schneider, Markus Glatzel, Berta Puig & Tim Magnus. (2020) Characterization of brain-derived extracellular vesicles reveals changes in cellular origin after stroke and enrichment of the prion protein with a potential role in cellular uptake. Journal of Extracellular Vesicles 9:1.
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Katrin Thüne, Matthias Schmitz, Anna Villar-Piqué, Hermann Clemens Altmeppen, Markus Schlomm, Saima Zafar, Markus Glatzel, Franc Llorens & Inga Zerr. (2019) The cellular prion protein and its derived fragments in human prion diseases and their role as potential biomarkers. Expert Review of Molecular Diagnostics 19:11, pages 1007-1018.
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Federico Benetti & Giuseppe Legname. (2015) New insights into structural determinants of prion protein folding and stability. Prion 9:2, pages 119-124.
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Articles from other publishers (20)

Ilaria Vanni, Floriana Iacobone, Claudia D’Agostino, Matteo Giovannelli, Laura Pirisinu, Hermann Clemens Altmeppen, Joaquin Castilla, Juan Maria Torres, Umberto Agrimi & Romolo Nonno. (2023) An optimized Western blot assay provides a comprehensive assessment of the physiological endoproteolytic processing of the prion protein. Journal of Biological Chemistry 299:2, pages 102823.
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Mohsin Shafiq, Stefano Da Vela, Ladan Amin, Neelam Younas, David A. Harris, Inga Zerr, Hermann C. Altmeppen, Dmitri Svergun & Markus Glatzel. (2022) The prion protein and its ligands: Insights into structure-function relationships. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1869:6, pages 119240.
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Valerija Kovač & Vladka Čurin Šerbec. (2022) Prion Protein: The Molecule of Many Forms and Faces. International Journal of Molecular Sciences 23:3, pages 1232.
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Doris Loh & Russel J. Reiter. (2022) Melatonin: Regulation of Prion Protein Phase Separation in Cancer Multidrug Resistance. Molecules 27:3, pages 705.
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Sungtae Yoon, Gyeongyun Go, Yeomin Yoon, Jiho Lim, Gaeun Lee & Sanghun Lee. (2021) Harnessing the Physiological Functions of Cellular Prion Protein in the Kidneys: Applications for Treating Renal Diseases. Biomolecules 11:6, pages 784.
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Berta Puig, Denise Yang, Santra Brenna, Hermann Clemens Altmeppen & Tim Magnus. (2020) Show Me Your Friends and I Tell You Who You Are: The Many Facets of Prion Protein in Stroke. Cells 9:7, pages 1609.
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Graham P. Roseman, Bei Wu, Mark A. Wadolkowski, David A. Harris & Glenn L. Millhauser. (2020) Intrinsic toxicity of the cellular prion protein is regulated by its conserved central region. The FASEB Journal 34:6, pages 8734-8748.
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Rosalina Gavín, Laia Lidón, Isidre Ferrer & José Antonio del Río. (2020) The Quest for Cellular Prion Protein Functions in the Aged and Neurodegenerating Brain. Cells 9:3, pages 591.
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Ajay Ashok, Min H. Kang, Aaron S. Wise, P. Pattabiraman, William M. Johnson, Michael Lonigro, Ranjana Ravikumar, Douglas J. Rhee & Neena Singh. (2019) Prion protein modulates endothelial to mesenchyme-like transition in trabecular meshwork cells: Implications for primary open angle glaucoma. Scientific Reports 9:1.
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Carolina Sánchez-López, Giulia Rossetti, Liliana Quintanar & Paolo Carloni. (2018) Structural Determinants of the Prion Protein N-Terminus and Its Adducts with Copper Ions. International Journal of Molecular Sciences 20:1, pages 18.
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Sarah Ulbrich, Petra Janning, Ralf Seidel, Jakob Matschke, Anika Gonsberg, Sebastian Jung, Markus Glatzel, Martin Engelhard, Konstanze F. Winklhofer & Jörg Tatzelt. (2018) Alterations in the brain interactome of the intrinsically disordered N-terminal domain of the cellular prion protein (PrPC) in Alzheimer’s disease. PLOS ONE 13:5, pages e0197659.
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Luise Linsenmeier, Hermann C. Altmeppen, Sebastian Wetzel, Behnam Mohammadi, Paul Saftig & Markus Glatzel. (2017) Diverse functions of the prion protein – Does proteolytic processing hold the key?. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1864:11, pages 2128-2137.
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Giuseppe Legname. (2017) Elucidating the function of the prion protein. PLOS Pathogens 13:8, pages e1006458.
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Abhishek Asthana, Shounak Baksi, Ajay Ashok, Shilpita Karmakar, Najiba Mammadova, Robyn Kokemuller, Mary Heather Greenlee, Qingzhong Kong & Neena Singh. (2017) Prion protein facilitates retinal iron uptake and is cleaved at the β-site: Implications for retinal iron homeostasis in prion disorders. Scientific Reports 7:1.
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Ilaria Vanni, Sergio Migliore, Gian Mario Cosseddu, Michele Angelo Di Bari, Laura Pirisinu, Claudia D’Agostino, Geraldina Riccardi, Umberto Agrimi & Romolo Nonno. (2016) Isolation of a Defective Prion Mutant from Natural Scrapie. PLOS Pathogens 12:11, pages e1006016.
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Silvia Vilches, Cristina Vergara, Oriol Nicolás, Ágata Mata, José A. del Río & Rosalina Gavín. (2015) Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells. Molecular Neurobiology 53:7, pages 4438-4448.
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Ladan Amin, Xuan T. A. Nguyen, Irene Giulia Rolle, Elisa D'Este, Gabriele Giachin, Thanh Hoa Tran, Vladka Čurin Šerbec, Dan Cojoc & Giuseppe Legname. (2016) Characterization of prion protein function by focal neurite stimulation. Journal of Cell Science.
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Maren K. Bakkebø, Sophie Mouillet-Richard, Arild Espenes, Wilfred Goldmann, Jörg Tatzelt & Michael A. Tranulis. (2015) The Cellular Prion Protein: A Player in Immunological Quiescence. Frontiers in Immunology 6.
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Nunzio Iraci, Claudia Stincardini, Maria Letizia Barreca & Emiliano Biasini. (2015) Decoding the function of the N-terminal tail of the cellular prion protein to inspire novel therapeutic avenues for neurodegenerative diseases. Virus Research 207, pages 62-68.
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Federico Benetti, Xevi Biarnés, Francesco Attanasio, Gabriele Giachin, Enrico Rizzarelli & Giuseppe Legname. (2014) Structural Determinants in Prion Protein Folding and Stability. Journal of Molecular Biology 426:22, pages 3796-3810.
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