Affiliation: Istituto di Ricerche Farmacologiche Mario Negri
- Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion diseaseRoberto Chiesa
Dulbecco Telethon Institute and Department of Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri, 20157 Milan, Italy
Proc Natl Acad Sci U S A 102:238-43. 2005..These results provide insights into the pathogenesis of prion diseases and have important implications for the treatment of these disorders...
- Neurotoxic and gliotrophic activity of a synthetic peptide homologous to Gerstmann-Sträussler-Scheinker disease amyloid proteinLuana Fioriti
Istituto di Ricerche Farmacologiche Mario Negri, 20157 Milano, Italy
J Neurosci 27:1576-83. 2007..Prnp0/0 astrocytes were insensitive to the proliferative stimulus of PrP 82-146. These results underline the role of cerebral accumulation of abnormally folded PrP fragments and indicate that cellular PrP governs the pathogenic process...
- Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalitiesRoberto Chiesa
Department of Neuroscience, Dulbecco Telethon Institute, Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy
J Neurosci 28:13258-67. 2008....
- Analysis of the cerebellar proteome in a transgenic mouse model of inherited prion disease reveals preclinical alteration of calcineurin activityEmiliano Biasini
Prion Unit, Dulbecco Telethon Institute, Istituto di Ricerche Farmacologiche Mario Negri, Via Eritrea, Milano, Italy
Proteomics 6:2823-34. 2006....
- The neurotoxicity of prion protein (PrP) peptide 106-126 is independent of the expression level of PrP and is not mediated by abnormal PrP speciesLuana Fioriti
Dulbecco Telethon Institute, Istituto di Ricerche Farmacologiche Mario Negri, Milan 20157, Italy
Mol Cell Neurosci 28:165-76. 2005..These results indicate that PrP106-126 interferes with a PrP function that requires only low protein levels, and is not impaired by a pathogenic insertion in the octapeptide region...
- Cytosolic prion protein (PrP) is not toxic in N2a cells and primary neurons expressing pathogenic PrP mutationsLuana Fioriti
Dulbecco Telethon Institute (DTI) and Department of Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri, Milano 20157, Italy
J Biol Chem 280:11320-8. 2005....
- Role of plasminogen in propagation of scrapieMario Salmona
Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy
J Virol 79:11225-30. 2005..In conclusion, our data indicate that plasminogen has no major effect on the survival of scrapie agent-infected mice...
- Specific recognition of biologically active amyloid-β oligomers by a new surface plasmon resonance-based immunoassay and an in vivo assay in Caenorhabditis elegansMatteo Stravalaci
Department of Molecular Biochemistry and Pharmacology, Istituto di Ricerche Farmacologiche Mario Negri, 20156 Milan, Italy
J Biol Chem 287:27796-805. 2012..The SPR-based immunoassay provides new opportunities for detection of toxic Aβ oligomers in biological samples and could be adapted to study misfolding proteins in other neurodegenerative disorders...
- Synthetic miniprion PrP106Valentina Bonetto
Dulbecco Telethon Institute, Department of Molecular Biochemistry and Pharmacology, Istituto di Ricerche Farmacologiche Mario Negri, Via Eritrea 62, Milan 20157, Italy
J Biol Chem 277:31327-34. 2002..These features are central properties of PrP(Sc) and make sPrP106 an excellent tool for investigating the molecular basis of the conformational conversion of PrP(C) into PrP(Sc) and prion disease pathogenesis...
- Molecular distinction between pathogenic and infectious properties of the prion proteinRoberto Chiesa
Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri 63110, USA
J Virol 77:7611-22. 2003..Our analysis provides new molecular insight into an emerging puzzle in prion biology, the discrepancy between the infectious and neurotoxic properties of PrP...
- A murine model of a familial prion diseaseDavid A Harris
Department of Cell Biology and Physiology, Washington University School of Medicine, 660 South Euclid Ave, St Louis, MO 63110, USA
Clin Lab Med 23:175-86. 2003..Because the mutant protein in the mice is highly neurotoxic but appears to lack infectivity, further analysis of its properties promises to shed new light on the molecular distinction between pathogenic and infectious forms of PrP...
- Multiple biochemical similarities between infectious and non-infectious aggregates of a prion protein carrying an octapeptide insertionEmiliano Biasini
Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, Missouri 63110, USA
J Neurochem 104:1293-308. 2008..Collectively, these results suggest that infectious and non-infectious aggregates of PrP share common structural features accounting for their toxicity, and that self-propagation of PrP involves more subtle molecular differences...
- Analysis of mammalian scrapie protein by novel monoclonal antibodies recognizing distinct prion protein glycoforms: an immunoblot and immunohistochemical study at the light and electron microscopic levelsAndrea Matucci
Section of Immunology, Department of Pathology, University of Verona, Policlinico G.B. Rossi, P. le L.A. Scuro 10, 37134 Verona, Italy
Brain Res Bull 65:155-62. 2005..These novel anti-PrP mAbs provide tools to investigate the subcellular site of PrP deposition in mammalian prion diseases and may also contribute to assess the role of different PrP glycoforms in human and animal prion diseases...
- Conditions of endoplasmic reticulum stress favor the accumulation of cytosolic prion proteinAndrea Orsi
Universita Vita Salute San Raffaele, DiBiT Istituto Scientifico San Raffaele, Via Olgettina 58, 20132 Milano, Italy
J Biol Chem 281:30431-8. 2006..These studies reveal a link between ER stress and the formation of cytosolic PrP isoforms potentially endowed with novel signaling or cytotoxic functions...
- Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125Aimin Li
Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
EMBO J 26:548-58. 2007..We speculate that altered binding interactions involving the 105-125 region of PrP may also play a role in generating neurotoxic signals during prion infection...
- Osteointegration of titanium and hydroxyapatite rough surfaces in healthy and compromised cortical and trabecular bone: in vivo comparative study on young, aged, and estrogen-deficient sheepVeronica Borsari
Laboratory of Experimental Surgery, Research Institute Codivilla Putti, Rizzoli Orthopaedic Institute, Bologna, Italy
J Orthop Res 25:1250-60. 2007..9%) with no significant differences between groups. In conclusion, the performance of TI01 and HT01 surfaces was high not only in YOUNG, but also in OVX animals and, therefore, they might be useful for aged and osteoporotic patients...
- Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradationBettina Drisaldi
Department of Cell Biology and Physiology, Washington University School of Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA
J Biol Chem 278:21732-43. 2003..Our results clarify the role of the proteasome in the cell biology of PrP, and suggest reasonable hypotheses for the molecular pathology of inherited prion diseases...