Hummler, E., Cole, T. J., Blendy, J. A., Ganss, R., Aguzzi, A., Schmid, W., ...
The cAMP response element binding protein (CREB) has been implicated as a key regulator in the transcriptional control of many genes. To assess the functional importance of CREB in vivo and its role...
Tyrosinase is a new marker for cell populations in the mouse neural tube (2008)
Tief, K., Schmidt, A., Aguzzi, A., Beermann, F.
Tyrosinase, the key enzyme in melanin synthesis, is expressed in pigment cells derived from both neural crest and neuroectoderm. The present study was performed to detect tyrosinase promoter activity...
Mice devoid of PrP are resistant to scrapie (2007)
Bueler, H., Aguzzi, A., Sailer, A., Greiner, R. A., Autenried, P., Aguet, M., ...
S.B. Prusiner proposed that the infectious agent of scraple, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior....
Role of Prp in Prion Diseases (2007)
Weissmann, C., Bueler, H., Sailer, A., Fischer, M., Aguet, M., Aguzzi, A.
PrP-deficient mice are resistant to scrapie (2007)
Weissmann, C., Bueler, H., Fischer, M., Sailer, A., Aguzzi, A., Aguet, M.
Prusiner proposed that the infectious agent of scrapie, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior. When...
Deficient signaling in mice devoid of double-stranded RNA-dependent protein kinase (2007)
Yang, Y. L., Reis, L. F., Pavlovic, J., Aguzzi, A., Schafer, R., Kumar, A., ...
Double-stranded RNA-dependent protein kinase (PKR) has been implicated in interferon (IFN) induction, antiviral response and tumor suppression. We have generated mice devoid of functional PKR (Pkr%)....
Lethal recessive myelin toxicity of prion protein lacking its central domain. (2007)
Baumann, F, Tolnay, M, Brabeck, C, Pahnke, J, Kloz, U, Niemann, H H, ...
PrP(C)-deficient mice expressing prion protein variants with large amino-proximal deletions (termed PrP(DeltaF)) suffer from neurodegeneration, which is rescued by full-length PrP(C). We now report...
Insights into prion strains and neurotoxicity. (2007)
Aguzzi, A, Heikenwalder, M, Polymenidou, M
Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are caused by prions and affect humans and many animal species. It is now widely accepted that the infectious...
Expression of lymphotoxin beta governs immunity at two distinct levels. (2006)
Junt, T, Tumanov, A V, Harris, N, Heikenwalder, M, Zeller, N, Kuprash, D V, ...
Interaction of lymphotoxin alpha(1)beta(2) (LTalpha(1)beta(2)) with its receptor is key for the generation and maintenance of secondary lymphoid organ microstructure. We used mice conditionally...
Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis. (2006)
Neuronal vacuolation (spongiosis), neuronal death, and pronounced glial reactions are the hallmarks of transmissible spongiform encephalopathies (TSEs), or prion diseases. A wealth of physical,...
Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease. (2006)
Landolt, H P, Glatzel, M, Blättler, T, Achermann, P, Roth, C, Mathis, J, ...
BACKGROUND: The prevalence and characteristics of sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease (sCJD) are poorly understood. METHODS: Seven consecutive patients with definite sCJD...
Cerebrovascular P-glycoprotein expression is decreased in Creutzfeldt-Jakob disease. (2006)
Vogelgesang, S, Glatzel, M, Walker, L C, Kroemer, H K, Aguzzi, A, Warzok, R W
The abnormal conformation and assembly of proteins in the central nervous system is increasingly thought to be a critical pathogenic mechanism in neurodegenerative disorders such as Creutzfeldt-Jakob...
Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. (2006)
Schoch, G, Seeger, H, Bogousslavsky, J, Tolnay, M, Janzer, R C, Aguzzi, A, ...
BACKGROUND: Prion diseases are a group of invariably fatal neurodegenerative disorders affecting humans and a wide range of mammals. An essential part of the infectious agent, termed the prion, is...
Strain fidelity of chronic wasting disease upon murine adaptation. (2006)
Sigurdson, C J, Manco, G J, Schwarz, P, Liberski, P, Hoover, E A, Hornemann, S, ...
Chronic wasting disease (CWD), a prion disease of deer and elk, is highly prevalent in some regions of North America. The establishment of mouse-adapted CWD prions has proven difficult due to the...
Chronic wasting disease. (2006)
Until recently, chronic wasting disease of cervids, the only prion disease affecting wildlife, was believed to be geographically concentrated to Colorado and Wyoming within the United States....
Early and rapid engraftment of bone marrow-derived microglia in scrapie. (2006)
Priller, J, Prinz, M, Heikenwalder, M J, Zeller, N, Schwarz, P, Heppner, F L, ...
Holder of copyright: The Society for Neuroscience
Baenziger, S, Tussiwand, R, Schlaepfer, E, Mazzucchelli, L, Heikenwalder, M, Kurrer, M O, ...
Because of species selectivity, HIV research is largely restricted to in vitro or clinical studies, both limited in their ability to rapidly assess new strategies to fight the virus. To prospectively...
Pathogenesis of prion diseases: current status and future outlook. (2006)
The prion, a conformational variant of a host protein, is the infectious particle responsible for transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of humans and...
Prion infections, blood and transfusions. (2006)
Prion infections lead to invariably fatal diseases of the CNS, including Creutzfeldt-Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE), and scrapie in sheep. There have been...
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. (2005)
Polymenidou, M, Stoeck, K, Glatzel, M, Vey, M, Bellon, A, Aguzzi, A
BACKGROUND: The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform ratio of protease-resistant prion protein (PrP(Sc)), and on PRNP haplotype. On...
Galvan, C, Camoletto, P G, Dotti, C G, Aguzzi, A, Ledesma, M D
Defects in cellular localization and trafficking seem to facilitate the conversion of PrP(C) into the disease-associated form, PrP(Sc). Still, it is not clear to which membrane compartments PrP(C)...
Genetic prion disease: the EUROCJD experience. (2005)
Kovács, G G, Puopolo, M, Ladogana, A, Pocchiari, M, Budka, H, Van Duijn, C M, ...
A total of 10-15% of human transmissible spongiform encephalopathies (TSEs) or prion diseases are characterised by disease-specific mutations in the prion protein gene (PRNP). We examined the...
Coincident scrapie infection and nephritis lead to urinary prion excretion. (2005)
Seeger, H, Heikenwalder, M, Zeller, N, Kranich, J, Schwarz, Petra, Gaspert, A, ...
Prion infectivity is typically restricted to the central nervous and lymphatic systems of infected hosts, but chronic inflammation can expand the distribution of prions. We tested whether chronic...
Gavín, R, Braun, Nathalie, Nicolas, O, Parra, B, Ureña, J M, Mingorance, A, ...
Prion diseases are characterised by severe neural lesions linked to the presence of an abnormal protease-resistant isoform of cellular prion protein (PrPc). The peptide PrP(106-126) is widely used as...
The prion gene is associated with human long-term memory. (2005)
Papassotiropoulos, A, Wollmer, M A, Aguzzi, A, Hock, Christoph, Nitsch, R M
Human cognitive processes are highly variable across individuals and are influenced by both genetic and environmental factors. Although genetic variations affect short-term memory in humans, it is...
Paracrine inhibition of prion propagation by anti-PrP single-chain Fv miniantibodies. (2005)
Donofrio, G, Heppner, F L, Polymenidou, M, Musahl, C, Aguzzi, A
Prion diseases are characterized by the deposition of PrP(Sc), an abnormal form of the cellular prion protein PrP(C). A growing body of evidence suggests that antibodies to PrP(C) can antagonize...
No influence of amyloid-beta-degrading neprilysin activity on prion pathogenesis. (2005)
Glatzel, M, Mohajeri, M H, Poirier, R, Nitsch, R M, Schwarz, Petra, Lu, B, ...
Transmissible spongiform encephalopathies are characterized by the accumulation of PrPSc, a protease-resistant form of a host-derived protein termed PrPC. Substantial evidence indicates that PrPSc...
Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC. (2005)
Radovanovic, I, Braun, Nathalie, Giger, O T, Mertz, K, Miele, G, Prinz, M, ...
The cellular prion protein PrP(C) confers susceptibility to transmissible spongiform encephalopathies, yet its normal function is unknown. Although PrP(C)-deficient mice develop and live normally,...
Ladogana, A, Puopolo, M, Croes, E A, Budka, H, Jarius, C, Collins, S, ...
BACKGROUND: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the...
An essential function for NBS1 in the prevention of ataxia and cerebellar defects. (2005)
Frappart, P O, Tong, W M, Demuth, I, Radovanovic, I, Herceg, Z, Aguzzi, A, ...
Nijmegen breakage syndrome (NBS), ataxia telangiectasia and ataxia telangiectasia-like disorder (ATLD) show overlapping phenotypes such as growth retardation, microcephaly, cerebellar developmental...
Huber, C, Thielen, C, Seeger, H, Schwarz, Petra, Montrasio, F, Wilson, M R, ...
Affinity maturation and Ab class switches occur in lymphoid germinal centers (GCs), in which differentiation and maintenance depend on lymphotoxin (LT) signaling and include differentiation of...
Human prion diseases: molecular and clinical aspects. (2005)
Glatzel, M, Stoeck, K, Seeger, H, Lührs, T, Aguzzi, A
Compared with that of other human pathogens, the proposed replicative cycle of prions is disarmingly simple. It encompasses misfolding of a single protein, the cellular prion protein (PrPC), into a...
Nikles, D, Bach, P, Boller, K, Merten, C A, Montrasio, F, Heppner, F L, ...
Passive immunization with antibodies directed against the cellular form of the prion protein (PrPC) can protect against prion disease. However, active immunization with recombinant prion protein has...
Russmann, H, Vingerhoets, F, Miklossy, J, Maeder, P, Glatzel, M, Aguzzi, A, ...
To investigate a possible relationship between the severity of pathological and radiological lesions in diffusion-weighted MRI (DWI) we compared DWI findings from 6 sequential brain MRI scans with...
A pathogenic PrP mutation and Doppel interfere with polarized sorting of the prion protein (2005)
Uelhoff,A., Tatzelt,J., Aguzzi,A., Winklhofer,K. F., Haass,C.
Chronic lymphocytic inflammation specifies the organ tropism of prions. (2005)
Heikenwalder, M, Zeller, N, Seeger, H, Prinz, M, Klöhn, P C, Schwarz, Petra, ...
Prions typically accumulate in nervous and lymphoid tissues. Because proinflammatory cytokines and immune cells are required for lymphoid prion replication, we tested whether inflammatory conditions...
A pathogenic PrP mutation and doppel interfere with polarized sorting of the prion protein. (2005)
Uelhoff, A, Tatzelt, J, Aguzzi, A, Winklhofer, K F, Haass, C
Several proteins linked to neurodegenerative diseases, such as the beta-amyloid precursor protein, amyloid beta-peptide, beta-secretase, and tau, undergo selective polarized sorting. We investigated...
Masel, J, Genoud, Nicolas, Aguzzi, A
Soluble dimeric prion protein (PrP-Fc(2)) binds to the disease-associated prion protein PrP(Sc), and inhibits prion replication when expressed in transgenic mice. Prion inhibition is effective even...
Experimental autoimmune encephalomyelitis repressed by microglial paralysis. (2005)
Heppner, F L, Greter, M, Marino, D, Falsig, J, Raivich, G, Hövelmeyer, N, ...
Although microglial activation occurs in inflammatory, degenerative and neoplastic central nervous system (CNS) disorders, its role in pathogenesis is unclear. We studied this question by generating...
Steinacker, P, Schwarz, P, Reim, K, Brechlin, Peter, Jahn, O, Kratzin, H, ...
The diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) is based on typical clinical findings and is supported by a positive 14-3-3 Western blot of cerebrospinal fluid. However, it is not clear...
Approaches to therapy of prion diseases. (2005)
Devising approaches to the therapy of transmissible spongiform encephalopathies, or prion diseases, is beset by many difficulties. For one, the nature of the infectious agent, the prion, is...
A pathogenic PrP mutation and Doppel interfere with polarized sorting of the prion protein (2005)
Uelhoff, A., Tatzelt, J., Aguzzi, A., Winklhofer, K. F., Haass, C.
Reconstructing prions: fibril assembly from simple yeast to complex mammals. (2005)
Sigurdson, C B, Polymenidou, M, Aguzzi, A
With the epizootics of bovine spongiform encephalopathy (BSE) in North American cattle, BSE infections in goats, new forms of human Creutzfeldt-Jakob disease (CJD) and the spread of chronic wasting...
PrPSc in mammary glands of sheep affected by scrapie and mastitis. (2005)
Ligios, C B, Sigurdson, C W, Santucciu, C, Carcassola, G, Manco, G, Basagni, M, ...
Hugel, B, Martínez, M C, Kunzelmann, C, Blättler, T, Aguzzi, A, Freyssinet, J M
Because expressed at a significant level at the membrane of human T cells, we made the hypothesis that the cellular prion protein (PrPc) could behave as a receptor, and be responsible for signal...
Spaeth, N, Wyss, M T, Weber, B, Scheidegger, S, Lutz, A, Verwey, J, ...
Differentiation between posttherapy radiation necrosis and recurrent tumor in humans with brain tumor is still a difficult diagnostic task. The new PET tracers (18)F-fluoro-ethyl-l-tyrosine (FET) and...
Polymenidou, M, Heppner, F L, Pellicioli, E C, Urich, E, Miele, G, Braun, Nathalie, ...
Prion diseases are characterized by the deposition of an abnormal form (termed PrP(Sc)) of the cellular prion protein (PrP(C)). Because antibodies to PrP(C) can antagonize deposition of PrP(Sc) in...
Recent developments in prion immunotherapy. (2004)
Antibody-based immunotherapy may represent a realistic approach against prion diseases, given that antibodies to the cellular prion protein PrPC have been shown to antagonize deposition of the...
Pocchiari, M, Puopolo, M, Croes, E A, Budka, H, Gelpi, E, Collins, S, ...
A collaborative study of human transmissible spongiform encephalopathies has been carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this...
Antiprion immunotherapy: to suppress or to stimulate? (2004)
Although human prion diseases are rare, they are invariably fatal, and treatments remain elusive. Hundreds of iatrogenic prion transmissions have occurred in the past two decades, and the bovine...
The AP-1 transcription factor c-Jun is required for efficient axonal regeneration. (2004)
Raivich, G, Bohatschek, M, Da Costa, C, Iwata, O, Galiano, M, Hristova, M, ...
Nerve injury triggers numerous changes in the injured neurons and surrounding nonneuronal cells that ultimately result in successful target reinnervation or cell death. c-Jun is a component of the...
The AP-1 transcription factor c-Jun is required for efficient axonal regeneration (2004)
Raivich, G., Bohatschek, M., Da Costa, C., Iwata, O., Galiano, M., Hristova, M., ...
Nerve injury triggers numerous changes in the injured neurons and surrounding nonneuronal cells that ultimately result in successful target reinnervation or cell death. c-Jun is a component of the...
Progress and problems in the biology, diagnostics, and therapeutics of prion diseases. (2004)
Aguzzi, A, Heikenwalder, M, Miele, G
The term "prion" was introduced by Stanley Prusiner in 1982 to describe the atypical infectious agent that causes transmissible spongiform encephalopathies, a group of infectious neurodegenerative...
Intrinsic resistance of oligodendrocytes to prion infection. (2004)
Prinz, M, Montrasio, F, Furukawa, H, Van Der Haar, M E, Schwarz, Petra, Rülicke, T, ...
Within the CNS, the normal form of cellular prion protein (PrP(C)) is expressed on neurons, oligodendrocytes, and astrocytes. The contribution of these cell types to prion replication and...
The peripheral nervous system and the pathogenesis of prion diseases. (2004)
Glatzel, M, Giger, Olivier, Braun, Nathalie, Aguzzi, A
Prion diseases are inevitably fatal neurodegenerative conditions which affect humans and a wide variety of animals. Unlike other protein aggregation diseases such as Alzheimer's, Parkinson's, and...
IL-6 is required for glioma development in a mouse model. (2004)
Weissenberger, J, Loeffler, S, Kappeler, A, Kopf, M, Lukes, A, Afanasieva, T A, ...
The pleiotropic cytokine interleukin-6 (IL-6) contributes to malignant progression and apoptosis resistance of various cancer types. Although IL-6 is elevated in malignant gliomas, and glioma cells...
Disruption of Doppel prevents neurodegeneration in mice with extensive Prnp deletions. (2004)
Genoud, Nicolas, Behrens, A, Miele, G, Robay, D, Heppner, F L, Freigang, S, ...
The Prnp gene encodes the cellular prion protein PrP(C). Removal of its ORF does not result in pathological phenotypes, but deletions extending into the upstream intron result in cerebellar...
Alzheimer's Abeta vaccination of rhesus monkeys (Macaca mulatta). (2004)
Gandy, S, DeMattos, R B, Lemere, C A, Heppner, F L, Leverone, J, Aguzzi, A, ...
Recent preliminary data suggest that vaccination with Alzheimer's Abeta might reduce senile plaque load and stabilize cognitive decline in human Alzheimer's disease. To examine the mechanisms and...
Variant Creutzfeldt-jakob disease: between lymphoid organs and brain. (2004)
Glatzel, M, Giger, Olivier, Seeger, H, Aguzzi, A
Prion diseases are often caused by peripheral uptake of the infectious agent. To reach their ultimate target, the central nervous system (CNS), prions enter their host, replicate in lymphoid organs...
Heikenwalder, M, Polymenidou, M, Junt, T, Sigurdson, C J, Wagner, H, Akira, S, ...
DNA containing unmethylated cytidyl guanosyl (CpG) sequences, which are underrepresented in mammalian genomes but prevalent in prokaryotes, is endocytosed by cells of the innate immune system,...
Mammalian prion biology: one century of evolving concepts. (2004)
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions caused more than 250