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In Vivo Longitudinal 1H MRS Study of Transgenic Mouse Models of Prion Disease in the Hippocampus and Cerebellum at 14.1T

Cudalbu, Cristina ; Craveiro, Melanie ; Mlynárik, Vladimir ; Bremer, Juliane ; Aguzzi, Adriano ; Gruetter, Rolf

In: Neurochemical Research, 2015, vol. 40, no. 12, p. 2639-2646

A bispecific immunotweezer prevents soluble PrP oligomers and abolishes prion toxicity

Bardelli, Marco ; Frontzek, Karl ; Simonelli, Luca ; Hornemann, Simone ; Pedotti, Mattia ; Mazzola, Federica ; Carta, Manfredi ; Eckhardt, Valeria ; D’Antuono, Rocco ; Virgilio, Tommaso ; González, Santiago F. ; Aguzzi, Adriano ; Varani, Luca

In: Plos pathogens, 2018, vol. 14, no. 10, p. e1007335

Antibodies to the prion protein, PrP, represent a promising therapeutic approach against prion diseases but the neurotoxicity of certain anti-PrP antibodies has caused concern. Here we describe scPOM-bi, a bispecific antibody designed to function as a molecular prion tweezer. scPOM-bi combines the complementarity-determining regions of the neurotoxic antibody POM1 and the neuroprotective...

Peripheral administration of tau aggregates triggers intracerebral tauopathy in transgenic mice

Clavaguera, Florence ; Hench, Jürgen ; Lavenir, Isabelle ; Schweighauser, Gabriel ; Frank, Stephan ; Goedert, Michel ; Tolnay, Markus

In: Acta Neuropathologica, 2014, vol. 127, no. 2, p. 299-301

The protein disulfide isomerase ERp57 regulates the steady-state levels of the prion protein

Torres, Mauricio ; Medinas,Danilo B. ; Matamala, José Manuel ; Woehlbier, Ute ; Cornejo, Víctor Hugo ; Solda, Tatiana ; Andreu, Catherine ; Rozas, Pablo ; Matus, Soledad ; Muñoz, Natalia ; Vergara, Carmen ; Cartier, Luis ; Soto, Claudio ; Molinari, Maurizio ; Hetz, Claudio

In: The journal of biological chemistry, 2015, vol. 290, no. 39, p. 23631-23645

Although the accumulation of a misfolded and protease-resistant form of the prion protein (PrP) is a key event in Prion pathogenesis, the cellular factors involved in its folding and quality control are poorly understood. PrP is a glycosylated and disulfide-bonded protein synthesized at the endoplasmic reticulum (ER). The ER foldase ERp57 (also known as Grp58) is highly expressed in the brain...

Hereditary Systemic Angiopathy (HSA) with cerebral calcifications, retinopathy, progressive nephropathy, and hepatopathy

Winkler, D. ; Lyrer, P. ; Probst, A. ; Devys, D. ; Haufschild, T. ; Haller, S. ; Willi, N. ; Mihatsch, M. ; Steck, A. ; Tolnay, M.

In: Journal of Neurology, 2008, vol. 255, no. 1, p. 77-88

Abdominal seeding of an atypical teratoid/rhabdoid tumor of the pineal gland along a ventriculoperitoneal shunt catheter

Ingold, Barbara ; Moschopulos, Michael ; Hutter, Gregor ; Seeger, Harald ; Röthlisberger, Benno ; Landolt, Hans ; Yonekawa, Yasuhiro ; Jochum, Wolfram ; Heppner, Frank

In: Acta Neuropathologica, 2006, vol. 111, no. 1, p. 56-59

Neuropathological and molecular comparison between clinical and asymptomatic bovine spongiform encephalopathy cases

Sisó, Silvia ; Doherr, Marcus ; Botteron, Catherine ; Fatzer, Rosemarie ; Zurbriggen, Andreas ; Vandevelde, Marc ; Seuberlich, Torsten

In: Acta Neuropathologica, 2007, vol. 114, no. 5, p. 501-508

Polystyrene beads as an alternative support material for epitope identification of a prion-antibody interaction using proteolytic excision-mass spectrometry

Pimenova, Tatiana ; Meier, Lukas ; Roschitzki, Bernd ; Paraschiv, Gabriela ; Przybylski, Michael ; Zenobi, Renato

In: Analytical and Bioanalytical Chemistry, 2009, vol. 395, no. 5, p. 1395-1401

Current concepts and controversies in prion immunopathology

Heikenwalder, Mathias ; Prinz, Marco ; Heppner, Frank ; Aguzzi, Adriano

In: Journal of Molecular Neuroscience, 2004, vol. 23, no. 1-2, p. 3-11

A study of neurological diseases in farmed deer in Switzerland, with emphasis on chronic wasting disease

Sieber, Veronika ; Ryser-Degiorgis, Marie-Pierre ; Botteron, Catherine

In: European Journal of Wildlife Research, 2008, vol. 54, no. 2, p. 189-192