Giorgio Giaccone

Summary

Affiliation: Istituto Nazionale Neurologico Carlo Besta
Country: Italy

Publications

  1. pmc Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred
    G Giaccone
    Istituto Neurologico Carlo Besta, Milan, Italy
    Proc Natl Acad Sci U S A 89:9349-53. 1992
  2. pmc A novel phenotype of sporadic Creutzfeldt-Jakob disease
    G Giaccone
    Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, Milano 20133, Italy
    J Neurol Neurosurg Psychiatry 78:1379-82. 2007
  3. pmc Lipofuscin hypothesis of Alzheimer's disease
    Giorgio Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Palermo, Italy
    Dement Geriatr Cogn Dis Extra 1:292-6. 2011
  4. ncbi request reprint Tauopathy in human and experimental variant Creutzfeldt-Jakob disease
    G Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
    Neurobiol Aging 29:1864-73. 2008
  5. doi request reprint Neuropathology of the recessive A673V APP mutation: Alzheimer disease with distinctive features
    Giorgio Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Acta Neuropathol 120:803-12. 2010
  6. doi request reprint New mutations in MAPT gene causing frontotemporal lobar degeneration: biochemical and structural characterization
    Giacomina Rossi
    Division of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Neurobiol Aging 33:834.e1-6. 2012
  7. doi request reprint Variability of the clinical phenotype in an Italian family with dementia associated with an intronic deletion in the GRN gene
    Gabriella Marcon
    Division of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy DISM, University of Udine, Udine, Italy
    J Alzheimers Dis 26:583-90. 2011
  8. ncbi request reprint The epsilon isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease
    Giuseppe di Fede
    Department of Neuropathology and Neurology, Istituto Nazionale Neurologico Carlo Besta, Milan, Italy
    J Neuropathol Exp Neurol 66:124-30. 2007
  9. pmc Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
    Raffaella Capobianco
    Fondazione I R C C S Istituto Neurologico Carlo Besta, Milan, Italy
    PLoS Pathog 3:e31. 2007
  10. doi request reprint Hereditary cerebral hemorrhage with amyloidosis associated with the E693K mutation of APP
    Orso Bugiani
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11 20133, Milan, Italy
    Arch Neurol 67:987-95. 2010

Detail Information

Publications46

  1. pmc Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred
    G Giaccone
    Istituto Neurologico Carlo Besta, Milan, Italy
    Proc Natl Acad Sci U S A 89:9349-53. 1992
    ..These findings support the view that in the GSS Indiana kindred a stepwise degradation of PrP occurs in situ in the process of amyloid fibril formation...
  2. pmc A novel phenotype of sporadic Creutzfeldt-Jakob disease
    G Giaccone
    Istituto Nazionale Neurologico Carlo Besta, Via Celoria 11, Milano 20133, Italy
    J Neurol Neurosurg Psychiatry 78:1379-82. 2007
    ..These features define a novel neuropathological and molecular CJD phenotype...
  3. pmc Lipofuscin hypothesis of Alzheimer's disease
    Giorgio Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Palermo, Italy
    Dement Geriatr Cogn Dis Extra 1:292-6. 2011
    ..The presence of intraneuronal Aβ, similarities between AD and age-related macular degeneration, and the possible explanation of some of the unknown issues in AD suggest that this hypothesis should not be discarded out of hand...
  4. ncbi request reprint Tauopathy in human and experimental variant Creutzfeldt-Jakob disease
    G Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
    Neurobiol Aging 29:1864-73. 2008
    ..These findings suggest that the abnormal forms of PrP associated with vCJD trigger a tauopathy, and provide a paradigm for the early stages of tau pathology associated with cerebral amyloidoses, including Alzheimer disease...
  5. doi request reprint Neuropathology of the recessive A673V APP mutation: Alzheimer disease with distinctive features
    Giorgio Giaccone
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Acta Neuropathol 120:803-12. 2010
    ..Main peculiar features are the morphology, structural properties and composition of the Aβ deposits as well as their topographic distribution in the brain...
  6. doi request reprint New mutations in MAPT gene causing frontotemporal lobar degeneration: biochemical and structural characterization
    Giacomina Rossi
    Division of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Neurobiol Aging 33:834.e1-6. 2012
    ..Our findings highlight the importance of genetic analysis also in sporadic forms of FTLD, and the role of in vitro studies to evaluate the pathologic features of new mutations...
  7. doi request reprint Variability of the clinical phenotype in an Italian family with dementia associated with an intronic deletion in the GRN gene
    Gabriella Marcon
    Division of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy DISM, University of Udine, Udine, Italy
    J Alzheimers Dis 26:583-90. 2011
    ..Our data extend the phenotypic spectrum and the complexity of neurodegenerative diseases linked to GRN mutations...
  8. ncbi request reprint The epsilon isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease
    Giuseppe di Fede
    Department of Neuropathology and Neurology, Istituto Nazionale Neurologico Carlo Besta, Milan, Italy
    J Neuropathol Exp Neurol 66:124-30. 2007
    ..These results indicate that the epsilon isoform of 14-3-3 is a component of PrP amyloid deposits of GSS and suggest that this is the sole 14-3-3 isoform specifically involved in the neuropathologic changes associated with this disorder...
  9. pmc Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
    Raffaella Capobianco
    Fondazione I R C C S Istituto Neurologico Carlo Besta, Milan, Italy
    PLoS Pathog 3:e31. 2007
    ....
  10. doi request reprint Hereditary cerebral hemorrhage with amyloidosis associated with the E693K mutation of APP
    Orso Bugiani
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11 20133, Milan, Italy
    Arch Neurol 67:987-95. 2010
    ..To report the clinical, genetic, neuroimaging, and neuropathologic studies of patients with the hereditary cerebral hemorrhage with amyloidosis linked to the APP E693K mutation...
  11. doi request reprint APP mutations in the Aβ coding region are associated with abundant cerebral deposition of Aβ38
    Maria Luisa Moro
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Milan, Italy
    Acta Neuropathol 124:809-21. 2012
    ....
  12. doi request reprint Neocortical variation of Abeta load in fully expressed, pure Alzheimer's disease
    Chiara Cupidi
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
    J Alzheimers Dis 19:57-68. 2010
    ..This study may be relevant for the correct evaluation of therapeutic strategies for AD that specifically address Abeta pathology...
  13. doi request reprint A novel Italian presenilin 2 gene mutation with prevalent behavioral phenotype
    Gabriella Marcon
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
    J Alzheimers Dis 16:509-11. 2009
    ....
  14. doi request reprint Good gene, bad gene: new APP variant may be both
    Giuseppe di Fede
    Neurology V and Neuropathology, IRCCS Foundation Carlo Besta Neurological Institute, Via Celoria 11, 20133 Milan, Italy
    Prog Neurobiol 99:281-92. 2012
    ..Here we reviewed the studies on pathogenic mechanisms associated with the A673V mutation and the first experimental steps toward the development of a novel disease-modifying therapy for AD...
  15. doi request reprint MM2-thalamic Creutzfeldt-Jakob disease: neuropathological, biochemical and transmission studies identify a distinctive prion strain
    Fabio Moda
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Brain Pathol 22:662-9. 2012
    ..These data indicate that MM2-thalamic sCJD is caused by a prion strain distinct from the other sCJD subtypes including the MM2-cortical form...
  16. ncbi request reprint Sporadic Creutzfeldt-Jakob disease: the extent of microglia activation is dependent on the biochemical type of PrPSc
    Gianfranco Puoti
    Istituto Nazionale Neurologico Carlo Besta, Milano, and Dipartimento di Scienze Neurologiche, Seconda Universita di Napoli, Napoli, Italy
    J Neuropathol Exp Neurol 64:902-9. 2005
    ..These findings support the view that the phenotypic heterogeneity of sporadic CJD is largely determined by the physicochemical properties of distinct PrPSc conformers...
  17. ncbi request reprint Cognitive deficits in familial Alzheimer's disease associated with M239V mutation of presenilin 2
    Anna Rita Giovagnoli
    Division of Neuropathology and Neurology, Carlo Besta National Neurological Institute, Milan, Italy
    Dement Geriatr Cogn Disord 22:238-43. 2006
    ..Such grading of visuospatial, praxis, and language impairments highlights a distinctive pattern related to the M239V mutation of the presenilin 2 gene...
  18. doi request reprint A novel progranulin mutation causing frontotemporal lobar degeneration with heterogeneous phenotypic expression
    Giacomina Rossi
    Division of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
    J Alzheimers Dis 23:7-12. 2011
    ..Being predictive for GRN null mutations, plasma progranulin dosage should be included in diagnostic work-up of dementia...
  19. ncbi request reprint Neuropathological and clinical phenotype of an Italian Alzheimer family with M239V mutation of presenilin 2 gene
    Gabriella Marcon
    Istituto Nazionale Neurologico Carlo Besta, Milano, Italy
    J Neuropathol Exp Neurol 63:199-209. 2004
    ..These observations indicate that the Alzheimer kindred FLO10 associated with M239V mutation of presenilin 2 is characterized by some peculiarities of the clinical and neuropathologic phenotype compared to sporadic Alzheimer disease...
  20. doi request reprint Myoclonus in Creutzfeldt-Jakob disease: polygraphic and video-electroencephalography assessment of 109 patients
    Simona Binelli
    Unit of Neurophysiopathology, IRCCS Foundation C Besta Neurological Institute, Milan, Italy
    Mov Disord 25:2818-27. 2010
    ....
  21. ncbi request reprint A new function of microtubule-associated protein tau: involvement in chromosome stability
    Giacomina Rossi
    Department of Neuropathology, Neurological Institute Carlo Besta, Milano, Italy
    Cell Cycle 7:1788-94. 2008
    ..Our findings argue for a role of tau in chromosome stability by means of its interaction with both microtubules and chromatin...
  22. ncbi request reprint Periodic electroencephalogram complexes in a patient with variant Creutzfeldt-Jakob disease
    Simona Binelli
    National Neurological Institute Carlo Besta, Milan, Italy
    Ann Neurol 59:423-7. 2006
    ..To verify this point, we investigated the development of the EEG changes along the course of the disease in a pateint with vCJD...
  23. pmc Brain delivery of AAV9 expressing an anti-PrP monovalent antibody delays prion disease in mice
    Fabio Moda
    Division of Neuropathology and Neurology 5, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy
    Prion 6:383-90. 2012
    ..This approach is relevant for designing new therapeutic strategies for prion diseases and other disorders characterized by protein misfolding...
  24. doi request reprint APE1/Ref-1 in Alzheimer's disease: an immunohistochemical study
    Gabriella Marcon
    Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
    Neurosci Lett 466:124-7. 2009
    ..An increased nuclear expression of APE1/Ref-1 in AD cerebral cortex supports the view that the cellular adaptive response to the oxidative stress condition is involved in the pathogenesis of this disease...
  25. ncbi request reprint A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V
    F Tagliavini
    Istituto Nazionale Neurologico Carlo Besta, 20133 Milano, Italy
    J Biol Chem 276:6009-15. 2001
    ..Furthermore, the present data suggest that C-terminal fragments of PrP may participate in amyloid formation...
  26. ncbi request reprint Neuropathology of Gerstmann-Sträussler-Scheinker disease
    O Bugiani
    Istituto Neurologico Carlo Besta, 20133 Milano, Italy
    Microsc Res Tech 50:10-5. 2000
    ..The clinical and pathological variability observed in GSS families is related to both mutations and the M/V polymorphism at codon 129 of the mutated gene...
  27. ncbi request reprint Creutzfeldt-Jakob disease: Carnoy's fixative improves the immunohistochemistry of the proteinase K-resistant prion protein
    G Giaccone
    Istituto Neurologico Carlo Besta, Milano, Italy
    Brain Pathol 10:31-7. 2000
    ....
  28. ncbi request reprint Development of layer I of the human cerebral cortex after midgestation: architectonic findings, immunocytochemical identification of neurons and glia, and in situ labeling of apoptotic cells
    R Spreafico
    Department of Experimental Neurophysiology and Epileptology, Neurological Institute C Besta, Milan, Italy
    J Comp Neurol 410:126-42. 1999
    ..In addition, our findings argue against a disappearance of Cajal-Retzius cells due to regressive processes...
  29. pmc Role of plasminogen in propagation of scrapie
    Mario 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...
  30. pmc beta PP and Tau interaction. A possible link between amyloid and neurofibrillary tangles in Alzheimer's disease
    G Giaccone
    Divisione di Neuropatologia, Istituto Nazionale Neurologico Carlo Besta, Milano, Italy
    Am J Pathol 148:79-87. 1996
    ..These data suggest that beta PP or beta PP fragments containing the tau binding site are involved in the pathogenesis of PHFs in Alzheimer's disease...
  31. ncbi request reprint Hereditary and sporadic beta-amyloidoses
    Giuseppe di Fede
    Division of Neurology 5 Neuropathology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy
    Front Biosci (Landmark Ed) 18:1202-26. 2013
    ....
  32. pmc Tetracyclines affect prion infectivity
    Gianluigi Forloni
    Istituto di Ricerche Farmacologiche Mario Negri, Istituto Nazionale Neurologico Carlo Besta, and Istituto di Microbiologia e Immunologia Veterinaria, Universita degli Studi, 20100 Milano, Italy
    Proc Natl Acad Sci U S A 99:10849-54. 2002
    ..Our data suggest that these well characterized antibiotics reduce prion infectivity through a direct interaction with PrP(Sc) and are potentially useful for inactivation of BSE- or vCJD-contaminated products and prevention strategies...
  33. ncbi request reprint Spinocerebellar ataxia type 17 (SCA17): oculomotor phenotype and clinical characterization of 15 Italian patients
    Caterina Mariotti
    Division of Biochemistry and Genetics, Fondazione IRCCS Istituto Neurologico, C Besta, Milan, Italy
    J Neurol 254:1538-46. 2007
    ..Our clinical data confirm the large variability in SCA17 phenotypic presentation, and indicate that a peculiar combination of neuroradiological, electrophysiological and oculomotor findings is recognizable in SCA17...
  34. ncbi request reprint Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau
    O Bugiani
    Istituto Neurologico Carlo Besta, Milano, Italy
    J Neuropathol Exp Neurol 58:667-77. 1999
    ..Biochemically, recombinant tau protein with the P301S mutation showed a greatly reduced ability to promote microtubule assembly...
  35. doi request reprint Current concepts in Alzheimer's disease: a multidisciplinary review
    Ludovico Minati
    Science Direction Unit, Fondazione IRCCS Istituto Nazionale Neurologico Carlo Besta, Milano, Italy
    Am J Alzheimers Dis Other Demen 24:95-121. 2009
    ....
  36. ncbi request reprint A family with Alzheimer disease and strokes associated with A713T mutation of the APP gene
    G Rossi
    Division of Neuropathology and Neurology 5, Istituto Nazionale Neurologico Carlo Besta, Milan, Italy
    Neurology 63:910-2. 2004
    ..This indicates that the A713T mutation of the APP gene, lying at the gamma-secretase cleavage site, can be responsible for AD with symptomatic cerebral amyloid angiopathy...
  37. ncbi request reprint FVEPs in Creutzfeldt-Jacob disease: waveforms and interaction with the periodic EEG pattern assessed by single sweep analysis
    E Visani
    Department of Neurophysiology, C Besta National Neurological Institute, Via Celoria 11, 20133 Milan, Italy
    Clin Neurophysiol 116:895-904. 2005
    ..To characterise flash visual evoked potentials (FVEPs) in 20 patients with Creutzfeldt-Jacob disease (CJD), and assess the relationships between spontaneous EEG patterns and the responses to individual stimuli...
  38. doi request reprint Role of cMET expression in non-small-cell lung cancer patients treated with EGFR tyrosine kinase inhibitors
    P A Zucali
    Department of Medical Oncology and Hematology, Istituto Clinico Humanitas, Rozzano, Milan, Italy
    Ann Oncol 19:1605-12. 2008
    ..However, resistance mechanisms are not well understood. We evaluated several potential biological markers of intrinsic EGFR-TKIs-resistance in NSCLC...
  39. ncbi request reprint Immunohistochemical detection of nuclear survivin in NSCLC: a comparison of commercial antibodies
    B Vischioni
    Histopathology 50:671-5. 2007
  40. pmc Immunocytochemical detection of deoxycytidine kinase in haematological malignancies and solid tumours
    I Hubeek
    Department of Paediatric Haematology Oncology, VU University Medical Centre, De Boelelaan 1117, PO Box 7057, 1007 MB, Amsterdam, The Netherlands
    J Clin Pathol 58:695-9. 2005
    ..Deoxycytidine kinase (dCK) is responsible for the activation of several clinically important deoxynucleoside analogues used for the treatment of haematological and solid malignancies...
  41. ncbi request reprint Substitutions at codon 22 of Alzheimer's abeta peptide induce diverse conformational changes and apoptotic effects in human cerebral endothelial cells
    L Miravalle
    Department of Pathology, New York University School of Medicine, New York, New York 10016, USA
    J Biol Chem 275:27110-6. 2000
    ..The data suggest that different amino acids at position 22 confer distinct structural properties to the peptides that appear to influence the onset and aggressiveness of the disease rather than the phenotype...
  42. ncbi request reprint Medical treatment of non-small-cell lung cancer
    J Buter
    Department of Medical Oncology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
    Ann Oncol 16:ii229-32. 2005
  43. doi request reprint A novel PSEN2 mutation associated with a peculiar phenotype
    P Piscopo
    Istituto Superiore di Sanita, 00161 Rome, Italy
    Neurology 70:1549-54. 2008
    ..Mutations of presenilin 2 gene are a rare cause of familial Alzheimer disease (AD). We describe an Italian family with hereditary dementia associated with a novel mutation in the presenilin 2 gene...
  44. ncbi request reprint Creutzfeldt-Jakob disease with a novel extra-repeat insertional mutation in the PRNP gene
    V Pietrini
    Department of Neuroscience, Institute of Neurology, University of Parma, Italy
    Neurology 61:1288-91. 2003
    ..The different patterns of PrP deposition were associated with distinct types of protease-resistant PrP, similar to type 1 and type 2 of sCJD. The features suggest that this insertion is a pathogenic mutation...
  45. pmc Newer opportunities in systemic therapy of lung cancer
    A Rajan
    National Cancer Institute, Bethesda, MD, USA
    Ann Oncol 19:vii31-7. 2008
  46. ncbi request reprint The epidermal growth factor receptor pathway and its inhibition as anticancer therapy
    M L Janmaat
    Department of Medical Oncology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
    Drugs Today (Barc) 39:61-80. 2003
    ..These studies failed to demonstrate an increase in survival by adding ZD1839 together with chemotherapy in patients with advanced NSCLC...