Zhu Caihong, Li Zhihao, Li Bei, Pfammatter Manuela, Hornemann Simone, Aguzzi Adriano (2019), Unaltered prion disease in mice lacking developmental endothelial locus–1, in
Neurobiology of Aging, 76, 208-213.
Pease Daniel, Scheckel Claudia, Schaper Elke, Eckhardt Valeria, Emmenegger Marc, Xenarios Ioannis, Aguzzi Adriano (2019), Genome-wide identification of microRNAs regulating the human prion proteinBrain Pathology, in
Brain Pathology, 29(2), 232-244.
Liu Yingjun, Sorce Silvia, Nuvolone Mario, Domange Julie, Aguzzi Adriano (2018), Lymphocyte activation gene 3 (Lag3) expression is increased in prion infections but does not modify disease progression, in
Scientific Reports, 8(1), 14600-14600.
Liu Yingjun, Sorce Silvia, Nuvolone Mario, Domange Julie, Aguzzi Adriano (2018), Lymphocyte activation gene 3 (Lag3) expression is increased in prion infections but does not modify disease progression, in
Scientific Reports, 8(1), 14600-14600.
Reimann Regina R., Aguzzi Adriano (2018), Intrinsic Toxicity of Antibodies to the Globular Domain of the Prion Protein, in
Biological Psychiatry, 84(7), e51-e52.
Scheckel Claudia, Aguzzi Adriano (2018), Prions, prionoids and protein misfolding disorders, in
Nature Reviews Genetics, 19(7), 405-418.
Baral Pravas Kumar, Swayampakula Mridula, Aguzzi Adriano, James Michael N. G. (2018), Structural characterization of POM6 Fab and mouse prion protein complex identifies key regions for prions conformational conversion, in
The FEBS Journal, 285(9), 1701-1714.
Ackerman Sarah D., Luo Rong, Poitelon Yannick, Mogha Amit, Harty Breanne L., D’Rozario Mitchell, Sanchez Nicholas E., Lakkaraju Asvin K.K., Gamble Paul, Li Jun, Qu Jun, MacEwan Matthew R., Ray Wilson Zachary, Aguzzi Adriano, Feltri M. Laura, Piao Xianhua, Monk Kelly R. (2018), GPR56/ADGRG1 regulates development and maintenance of peripheral myelin, in
The Journal of Experimental Medicine, 215(3), 941-961.
Schütz Anne K., Hornemann Simone, Wälti Marielle A., Greuter Ladina, Tiberi Cinzia, Cadalbert Riccardo, Gantner Matthias, Riek Roland, Hammarström Per, Nilsson K. Peter R., Böckmann Anja, Aguzzi Adriano, Meier Beat H. (2018), Binding of Polythiophenes to Amyloids: Structural Mapping of the Pharmacophore, in
ACS Chemical Neuroscience, 9(3), 475-481.
Aguzzi Adriano, Lakkaraju Asvin K.K., Frontzek Karl (2018), Toward Therapy of Human Prion Diseases, in
Annual Review of Pharmacology and Toxicology, 58(1), 331-351.
Wulf Marie-Angela, Senatore Assunta, Aguzzi Adriano (2017), The biological function of the cellular prion protein: an update, in
BMC Biology, 15(1), 34-34.
Goniotaki Despoina, Lakkaraju Asvin K. K., Shrivastava Amulya N., Bakirci Pamela, Sorce Silvia, Senatore Assunta, Marpakwar Rajlakshmi, Hornemann Simone, Gasparini Fabrizio, Triller Antoine, Aguzzi Adriano (2017), Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity, in
PLOS Pathogens, 13(11), e1006733-e1006733.
Pfammatter Manuela, Andreasen Maria, Meisl Georg, Taylor Christopher G., Adamcik Jozef, Bolisetty Sreenath, Sánchez-Ferrer Antoni, Klenerman David, Dobson Christopher M., Mezzenga Raffaele, Knowles Tuomas P. J., Aguzzi Adriano, Hornemann Simone (2017), Absolute Quantification of Amyloid Propagons by Digital Microfluidics, in
Analytical Chemistry, 89(22), 12306-12313.
Aguzzi Adriano, Zhu Caihong (2017), Microglia in prion diseases, in
Journal of Clinical Investigation, 127(9), 3230-3239.
Ballmer Boris A., Moos Rita, Liberali Prisca, Pelkmans Lucas, Hornemann Simone, Aguzzi Adriano (2017), Modifiers of prion protein biogenesis and recycling identified by a highly parallel endocytosis kinetics assay, in
Journal of Biological Chemistry, 292(20), 8356-8368.
Nuvolone Mario, Paolucci Marta, Sorce Silvia, Kana Veronika, Moos Rita, Matozaki Takashi, Aguzzi Adriano (2017), Prion pathogenesis is unaltered in the absence of SIRPα-mediated "don't-eat-me" signaling, in
PLOS ONE, 12(5), e0177876-e0177876.
Leske Henning, Hornemann Simone, Herrmann Uli Simon, Zhu Caihong, Dametto Paolo, Li Bei, Laferriere Florent, Polymenidou Magdalini, Pelczar Pawel, Reimann Regina Rose, Schwarz Petra, Rushing Elisabeth Jane, Wüthrich Kurt, Aguzzi Adriano (2017), Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein, in
PLOS ONE, 12(2), e0170503-e0170503.
Nuvolone Mario, Schmid Nicolas, Miele Gino, Sorce Silvia, Moos Rita, Schori Christian, Beerli Roger R., Bauer Monika, Saudan Philippe, Dietmeier Klaus, Lachmann Ingolf, Linnebank Michael, Martin Roland, Kallweit Ulf, Kana Veronika, Rushing Elisabeth J., Budka Herbert, Aguzzi Adriano (2017), Cystatin F is a biomarker of prion pathogenesis in mice, in
PLOS ONE, 12(2), e0171923-e0171923.
Frontzek Karl, Pfammatter Manuela, Sorce Silvia, Senatore Assunta, Schwarz Petra, Moos Rita, Frauenknecht Katrin, Hornemann Simone, Aguzzi Adriano (2016), Neurotoxic Antibodies against the Prion Protein Do Not Trigger Prion Replication, in
PLOS ONE, 11(9), e0163601-e0163601.
Küffer Alexander, Lakkaraju Asvin K. K., Mogha Amit, Petersen Sarah C., Airich Kristina, Doucerain Cédric, Marpakwar Rajlakshmi, Bakirci Pamela, Senatore Assunta, Monnard Arnaud, Schiavi Carmen, Nuvolone Mario, Grosshans Bianka, Hornemann Simone, Bassilana Frederic, Monk Kelly R., Aguzzi Adriano (2016), The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6, in
Nature, 536(7617), 464-468.
Aguzzi Adriano, Altmeyer Matthias (2016), Phase Separation: Linking Cellular Compartmentalization to Disease, in
Trends in Cell Biology, 26(7), 547-558.
Theocharides A. P. A., Lundberg P., Lakkaraju A. K. K., Lysenko V., Myburgh R., Aguzzi A., Skoda R. C., Manz M. G. (2016), Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency, in
Blood, 127(25), 3253-3259.
Zhu Caihong, Herrmann Uli S., Falsig Jeppe, Abakumova Irina, Nuvolone Mario, Schwarz Petra, Frauenknecht Katrin, Rushing Elisabeth J., Aguzzi Adriano (2016), A neuroprotective role for microglia in prion diseases, in
The Journal of Experimental Medicine, 213(6), 1047-1059.
Nuvolone Mario, Hermann Mario, Sorce Silvia, Russo Giancarlo, Tiberi Cinzia, Schwarz Petra, Minikel Eric, Sanoudou Despina, Pelczar Pawel, Aguzzi Adriano (2016), Strictly co-isogenic C57BL/6J- Prnp−/− mice: A rigorous resource for prion science, in
The Journal of Experimental Medicine, 213(3), 313-327.
Reimann Regina R., Sonati Tiziana, Hornemann Simone, Herrmann Uli S., Arand Michael, Hawke Simon, Aguzzi Adriano (2016), Differential Toxicity of Antibodies to the Prion Protein, in
PLOS Pathogens, 12(1), e1005401-e1005401.
Aguzzi Adriano, Lakkaraju Asvin K.K. (2016), Cell Biology of Prions and Prionoids: A Status Report, in
Trends in Cell Biology, 26(1), 40-51.
Prion diseases are fatal neurodegenerative diseases, including Creutzfeldt-Jakob disease, which affect humans and other mammals. Due to the lack of knowledge of basic prion biology, no treatment options are currently available. We have previously focused on clarifying the physiological role of the N1 fragment of the prion protein (PrP) and exploiting a relatively new technique, cultured organotypic cerebellar slices (COCS), to study mechanisms of prion-induced cell death. To build upon this, we will continue to examine the importance of the N-terminus of PrP under physiological conditions, as well as in disease. Furthermore, we aim to understand the mechanism responsible for spongiosis, a hallmark observed in those affected by prion diseases. Aim1: Examine the role of the N-terminus in myelination Aim 2: Study the role of the N-terminus in familial prion diseasesAim 3: Examine the mechanism responsible for spongiosis in prion disease