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1Latest Papers from Table of Contents or Articles in PressJ. Schwartzentruber ; A. Korshunov ; X. Y. Liu ; D. T. Jones ; E. Pfaff ; K. Jacob ; D. Sturm ; A. M. Fontebasso ; D. A. Quang ; M. Tonjes ; V. Hovestadt ; S. Albrecht ; M. Kool ; A. Nantel ; C. Konermann ; A. Lindroth ; N. Jager ; T. Rausch ; M. Ryzhova ; J. O. Korbel ; T. Hielscher ; P. Hauser ; M. Garami ; A. Klekner ; L. Bognar ; M. Ebinger ; M. U. Schuhmann ; W. Scheurlen ; A. Pekrun ; M. C. Fruhwald ; W. Roggendorf ; C. Kramm ; M. Durken ; J. Atkinson ; P. Lepage ; A. Montpetit ; M. Zakrzewska ; K. Zakrzewski ; P. P. Liberski ; Z. Dong ; P. Siegel ; A. E. Kulozik ; M. Zapatka ; A. Guha ; D. Malkin ; J. Felsberg ; G. Reifenberger ; A. von Deimling ; K. Ichimura ; V. P. Collins ; H. Witt ; T. Milde ; O. Witt ; C. Zhang ; P. Castelo-Branco ; P. Lichter ; D. Faury ; U. Tabori ; C. Plass ; J. Majewski ; S. M. Pfister ; N. Jabado
Nature Publishing Group (NPG)
Published 2012Staff ViewPublication Date: 2012-01-31Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Adaptor Proteins, Signal Transducing/genetics ; Base Sequence ; Child ; Chromatin/*genetics/metabolism ; Chromatin Assembly and Disassembly/*genetics ; DNA Helicases/genetics ; DNA Mutational Analysis ; Exome/genetics ; Gene Expression Profiling ; Glioblastoma/*genetics ; Histones/*genetics/metabolism ; Humans ; Molecular Sequence Data ; Mutation/*genetics ; Nuclear Proteins/genetics ; Telomere/genetics ; Tumor Suppressor Protein p53/geneticsPublished by: -
2Latest Papers from Table of Contents or Articles in PressT. Sonati ; R. R. Reimann ; J. Falsig ; P. K. Baral ; T. O'Connor ; S. Hornemann ; S. Yaganoglu ; B. Li ; U. S. Herrmann ; B. Wieland ; M. Swayampakula ; M. H. Rahman ; D. Das ; N. Kav ; R. Riek ; P. P. Liberski ; M. N. James ; A. Aguzzi
Nature Publishing Group (NPG)
Published 2013Staff ViewPublication Date: 2013-08-02Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Amino Acid Sequence ; Animals ; Antibodies/*immunology/*toxicity ; Antibodies, Monoclonal/immunology/toxicity ; Binding Sites, Antibody ; Calpain/metabolism ; Cerebellum ; Creutzfeldt-Jakob Syndrome/metabolism ; Cross-Linking Reagents ; Epitope Mapping ; Female ; Immunoglobulin Fab Fragments/immunology/toxicity ; In Vitro Techniques ; Ligands ; Male ; Membrane Glycoproteins/metabolism ; Mice ; Molecular Sequence Data ; NADPH Oxidase/metabolism ; Neurodegenerative Diseases/metabolism ; Oxidative Stress ; *Pliability ; PrPC Proteins/chemistry/genetics/immunology ; Prions/*chemistry/genetics/*immunology ; Reactive Oxygen Species/metabolism ; Sequence Deletion/genetics ; Single-Chain Antibodies/immunology/toxicityPublished by: -
3Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Key words Tubulovesicular structures ; Prion protein ; Transmissible spongiform encephalopathy ; Immunogold ; electron microscopySource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Tubulovesicular structures (TVS) are disease-specific, intraneuronal particles found by thin-section electron microscopy in all of the transmissible spongiform encephalopathies. We used immunogold (both 10 nm immunogold and 1 nm immunogold silver enhanced) methods for ultrastructural localization of prion protein (PrP). In all scrapie models examined (263 K and 22CH in hamsters and 87V and ME7 in mice), TVS-containing processes were readily detected but neither these processes nor TVS themselves were decorated with gold particles. Even when amyloid plaques were observed in a close contact with TVS-containing neuronal processes, the processes remained unstained, while the plaques were decorated with gold particles. TVS located in areas adjacent to plaques in the 87V model and in areas of diffuse PrP immunolabelling in ME7 were also unlabelled with anti-PrP sera. Using immunogold techniques we were unable to label TVS with anti-PrP antibodies. As these technique proved to be sensitive enough to immunolabel not only amyloid plaques but also pre-amyloid accumulations of PrP, we strongly believe that the absence of staining reflects the structure of TVS and that they are not composed of PrP. That TVS are PrP negative may have several important implications for hypotheses about their nature. Principally, it does not support the suggestion that TVS are cross-sections of “thick tubules” visualized by touch-preparations of scrapie-affected mouse and hamster brains. If PrP is the infectious agent, as suggested by the prion hypothesis, the absence of stainable PrP in TVS would indicate that these are not the ultrastructural correlate of the agent. If, however, TVS turn out to be more than merely a useful ultrastructural marker for the whole group of transmissible spongiform encephalopathies, it may suggest that PrP and the agent are two separate entities.Type of Medium: Electronic ResourceURL: -
4Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Scrapie ; Dystrophic neurites ; VacuolationSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary Numbers of dystrophic neurites, seen with the electron microscope, in CA1 of the hippocampus of either C3H mice infected with 22C or 79A strains of scrapie, or LM mice infected with strain ME7 were greater than in age-matched control mice. Vacuolation, seen by light microscopy in CA1 of the hippocampus of mice infected with either 22C or 79A, preceded the increase in dystrophic neurites by up to about 20 days. In mice infected with ME7, however, the vacuolation followed the increase in dystrophic neurites by some 20 to 40 days. In view of the differences in the times at which dystrophic neurites and vacuolation were seen, no causative relationship between the two lesions appeared to exist.Type of Medium: Electronic ResourceURL: -
5Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Source: Springer Online Journal Archives 1860-2000Topics: MedicineType of Medium: Electronic ResourceURL: -
6Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Scrapie ; Creutzfeldt-Jakob disease ; Neuronal autophagy ; Neuropathology ; Electron microscopySource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary We report the presence of autophagic vacuoles (AV) in neuronal perikarya and neuronal processes of rodents with experimental scrapie and Creutzfeldt-Jakob disease. AV were composed of sequestrated cytoplasmic areas containing ribosomes and occasionally mitochondria and small secondary vacuoles. The formation of AV may contribute to neuronal degeneration and ultimately to neuronal loss.Type of Medium: Electronic ResourceURL: -
7Articles: DFG German National LicensesBarcikowska, M. ; Liberski, P. P. ; Boellaard, J. W. ; Brown, P. ; Gajdusek, D. C. ; Budka, H.
Springer
Published 1993Staff ViewISSN: 1432-0533Keywords: Amyloid ; Microglia ; Prion diseases ; Spongiform encephalopathies ; Gerstmann-Sträussler ; Scheinker syndromeSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary The microglial cell has been demonstrated as component of the cerebral amyloid plaque of Alzheimer's disease. Involvement of microglia in plaques of another cerebral amyloidosis, the Gerstmann-Sträussler-Scheinker syndrome (GSS), has found little attention. We examine here the presence of microglia in GSS plaques by immunohistochemistry and transmission electron microscopy. Paraffin sections from five brains of patients with GSS were immunolabelled with antibodies against prion protein, A4/β amyloid protein, ferritin, leukocyte common antigen, HLA-DR, CD 68, and the MAC387 epitope for microglia and monocytes/macrophages; microglia was also labelled with the Ricinus communis agglutinin-1 lectin. Such (immuno)labelling demonstrated many delicate cell processes and occasional somata within and around prion protein plaques in all GSS brains. Microglial immunoreactivity was strongest with anti-ferritin and variable with anti-macrophage antibodies. Ultrastructural examination of brain tissue from one autopsy and one biopsy of GSS identified microglial cells in close proximity of amyloid plaque fibrils. Our observations demonstrate microglia as an important component of the amyloid plaque in GSS and suggest a major role for microglia in processing and deposition, or at least organization, of prion protein amyloid. Thus, plaques in both transmissible and nontransmissible cerebral amyloidoses seem to develop via similar pathogenetic mechanisms, irrespective of differences in etiology and molecular composition of the amyloid.Type of Medium: Electronic ResourceURL: -
8Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Source: Springer Online Journal Archives 1860-2000Topics: MedicineType of Medium: Electronic ResourceURL: -
9Articles: DFG German National LicensesHainfellner, Johannes A. ; Wanschitz, J. ; Jellinger, Kurt ; Liberski, P. P. ; Gullotta, Filippo ; Budka, H.
Springer
Published 1998Staff ViewISSN: 1432-0533Keywords: Key words Alzheimer’s disease ; Creutzfeldt-Jakob ; disease ; Dementia ; Neuropathology ; Prion diseaseSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Creutzfeldt-Jakob disease (CJD) and Alzheimer’s disease (AD) share clinical, neuropathological, and pathogenetic features. To investigate eventual mutual influences, we screened prominently affected neocortex from 110 neuropathologically proven CJD patients for Alzheimer-type pathology with anti-β/A4, Bielschowsky and anti-tau (immuno)stains. The neuropathological classification of Alzheimer-type pathology was made according to the CERAD criteria. Results were controlled by comparison with Alzheimer-type changes in sections from the same cortical areas in 110 sex- and age-matched non-demented control patients. For comparison, the control patients were also classified according to the CERAD neuropathology criteria as if they had been demented. Alzheimer-type tissue changes as in definite and probable CERAD AD occur in 10.9% of the CJD patients and 19.1% of control patients (P = 0.11). The median age of CJD and control patients with CERAD AD is 72 and 68 years, respectively, which differs significantly from the median ages of 64 and 63 years, respectively, in the non-AD/CJD and non-AD control patients. Since CERAD criteria include “presence of other neuropathological lesions likely to cause dementia”, an AD diagnosis in CJD patients (all of whom are demented) is solely based on densities of neuritic plaques. Similar Alzheimer-type changes in even higher frequency, however, are also present in elderly non-demented controls. Thus, the coexistence of Alzheimer-type pathology in CJD most likely represents an age-related change. Deposits of prion protein (PrP) frequently accumulate at the periphery of β/A4 plaques. The presence of β/A4 amyloid in the brain may influence PrP morphogenesis.Type of Medium: Electronic ResourceURL: -
10Articles: DFG German National LicensesGuiroy, D. C. ; Liberski, P. P. ; Alwasiak, J. ; Papierz, W. ; Gajdusek, D. C.
Springer
Published 1991Staff ViewISSN: 1432-0533Keywords: Amyloid β-protein ; Progressive dementia ; Cerebellar plaquesSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary We report the immunolocalization of extensive amyloid β-protein in senile plaques, cerebrovascular amyloid deposits, neurofibrillary tangles and preamyloid in a 32-year-old man with progressive dementia not to trisomy 21 or trauma. These amyloid deposits were non-reactive to antibodies directed against scrapie amyloid. Our data indicate that the presence of amyloid β-protein is not limited to normal aging, Alzheimer's disease and related disorders but is also found in younger individuals with progressive dementia.Type of Medium: Electronic ResourceURL: -
11Articles: DFG German National LicensesLiberski, P. P. ; Barcikowska, M. ; Cervenakova, L. ; Bratosiewicz, J. ; Marczewska, M. ; Brown, P. ; Gajdusek, D. C.
Springer
Published 1998Staff ViewISSN: 1432-0533Keywords: Key words Prions ; Creutzfeldt-Jakob disease ; Gerstmann-Sträussler-Scheinker diseaseSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract We report here an unusual sporadic case of Creutzfeldt-Jakob disease (CJD) characterized by an abundance of prion protein (PrP)-immunopositive kuru and multicentric but not florid plaques. Molecular genetic analysis of the PRNP open reading frame region spanning codons 8–221 was performed. Neither deletion nor insertion mutations were detected in the repeat area of the PRNP. No pathogenic mutation was found in the sequenced region between codon 108–221. Restriction analysis of the amplified fragment using restriction endonucleases DdeI, PvuII and AluI did not show any of the previously described pathogenic mutations at codon 102, 105, and 117 associated with Gerstmann-Sträussler-Scheinker (GSS). The patient was heterozygous for the methionine/valine coding triplet at polymorphic codon 129 of the PRNP gene by sequence, restriction endonuclease analysis and hybridization with allele-specific nucleotides. Furthermore, hybridization with 32P-labeled allele-specific oligonucleotides confirmed the absence of pathogenic mutations at codons 102, 200 and 178. Such a case may present a missing “link” between sporadic CJD and familial GSS.Type of Medium: Electronic ResourceURL: -
12Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Creutzfeldt-Jakob disease ; Electron microscopy ; Scrapie ; Tubulovesicular structuresSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary We have consistently observed tubulovesicular structures in brain tissues during the terminal stages of naturally occurring and experimentally induced spongiform encephalopathies, irrespective of the host species and virus strain. In NIH Swiss mice inoculated intracerebrally or intraocularly with the Fujisaki strain of Creutzfeldt-Jakob disease (CJD) virus, tubulovesicular structures, measuring 20–50 nm in diameter, were particularly prominent in dilated, pre-and postsynaptic neuronal processes, occasionally being mixed with synaptic vesicles. These structures appeared 13 weeks following intracerebral inoculation, 5 weeks before the onset of clinical signs, when spongiform changes were also detected. The number and density of tubulovesicular structures increased steadily during the course of clinical disease, and were particularly abundant in mice 47 to 51 weeks after intraocular inoculation. In hamsters infected with the 263 K strain of scrapie virus, these structures were initially detected 3 weeks following intracerebral inoculation and increased dramatically at 10 weeks postinoculation. The appearance of tubulovesicular structures before the onset of overt disease in mice inoculated with CJD virus by either the intracerebral or intraocular route, and before the appearance of other neuropathological changes in hamsters infected with scrapie virus, indicate that they represent either a part or aggregate of the infectious virus or a pathological product of the infection.Type of Medium: Electronic ResourceURL: -
13Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Creutzfeldt ; Jakob disease ; Tubulovesicular structures ; Prion diseases ; Ultrastructure ; Transmissible spongiform encephalopathiesSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary By electron microscopy tubulovesicular structures (TVS) have been consistently observed in brain tissue of transmissible spongiform encephalopathies such as natural and experimental scrapie, bovine spongiform encephalopathy and experimentally induced, but not naturally occurring, Creutzfeldt-Jakob disease (CJD). For the first time we report here the presence of TVS in human brains with CJD as detected by transmission electron microscopy. TVS were observed in all three CJD specimens (two biopsies, one autopsy), but they were rare and were found only in one or two location(s) per grid. TVS were seen in distended pre- and postsynaptic terminals and measured approximately 35 nm in diameter; they were smaller and of higher electron density than synaptic vesicles. Their occurrence in all types of transmissible spongiform encephalopathies irrespective of the affected host and the strain of the infectious agent suggests their biological significance.Type of Medium: Electronic ResourceURL: -
14Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Creutzfeld ; Jakob disease ; White matter change ; Leukolysins ; Tumor necrosis factorSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary Creutzfeldt-Jakob disease (CJD), previously regarded as a neurodegenerative disorder strictly of the gray matter, occasionally occurs as a panencephalopathic form which is characterized by severe white matter damage. An ultrastructural study of the white matter pathology in mice experimentally infected with the Fujisaki strain of CJD virus revealed: (1) vacuoles within myelin sheaths, formed by splitting either at the major dense or intraperiod lines, or within axons; (2) macrophages filled with numerous myelin figures, lipid droplets and paracrystalline inclusions; (3) astrocytes actively digesting myelin debris; (4) unusual wrapping of several axons by a common myelin sheath; (5) vesicular degeneration of myelin sheaths; (6) close contact between numerous coated pits and outer myelin lamellae; and (7) proliferation of inner mesaxons. Our data indicate that the damage to myelinated axons in the panencephalopathic type of CJD is accomplished primarily by active degradation of myelin by macrophages and astrocytes and by formation of intra-axonal and intra-myelin vacuoles. The myelin vacuolation is most consistent with that produced by leukolysins released from activated macrophages and astrocytes.Type of Medium: Electronic ResourceURL: -
15Articles: DFG German National LicensesLiberski, P. P. ; Bratosiewicz, J. ; Barcikowska, M. ; Cervenakova, L. ; Marczewska, M. ; Brown, P. ; Gajdusek, D. C.
Springer
Published 2000Staff ViewISSN: 1432-0533Source: Springer Online Journal Archives 1860-2000Topics: MedicineType of Medium: Electronic ResourceURL: -
16Articles: DFG German National LicensesStaff View
ISSN: 0942-0940Keywords: Astrocytoma ; Ki-67 ; proliferating cell nuclear antigenSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary To compare the Ki-67 and Proliferating Cell Nuclear Antigen (PCNA), markers of cell proliferation, paraffin embedded surgical specimens from 56 human astrocytic tumours (8 pilocytic tumours, grade I; 9 low (II) grade and 9 anaplastic (grade III) astrocytomas and 30 glioblastomas, grade IV) were immunolabelled with the anti-PCNA (PC 10, DAKO) and anti-Ki-67 (DAKO) antibodies. For the latter immunostaining the microwave oven processing was performed. The Ki-67 and PCNA labelling indices (LIs) were statistically compared. For the majority of cases, PCNA LI was higher than that obtained with anti-Ki-67 antibody and the intensity of staining for PCNA was more variable. The statistically significant difference of the percentage of PCNA LIs was found only between low grade (I and II) and high grade (III and IV) tumours, while Ki-67 LIs discriminates each group of glial tumours; thus this latter marker is more sensitive and specific.Type of Medium: Electronic ResourceURL: -
17Articles: DFG German National LicensesStaff View
ISSN: 1432-8798Source: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary We studied the targeting of spongiform lesions within the visual pathways after intraocular injection with the Fujisaki strain of Creutzfeldt-Jakob disease (CJD) virus. The first lesions were observed 18 weeks postinoculation in the most superficial layer of the superior colliculus and in the lateral geniculate body contralateral to the side of the inoculation. Asymmetrical lesions in the superior colliculus were found also in mice sacrificed at 19, 22, and 27 weeks postinoculation. These results demonstrate that CJD virus spreads within the CNS via central axons of the visual pathways following intraocular inoculation.Type of Medium: Electronic ResourceURL: -
18Articles: DFG German National LicensesMinagawa, H. ; Mora, C. A. ; Asher, D. M. ; Stone, G. A. ; Liberski, P. P. ; Gibbs, C. J.
Springer
Published 1991Staff ViewISSN: 1432-8798Source: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary Rabbits were infected successfully with two strains of human T-cell leukemia virus type I (HTLV-I), one isolated from a Colombian patient with HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) and the other from an asymptomatic carrier. HTLV-I was repeatedly demonstrated in peripheral blood mononuclear cells (PBMNC) of infected rabbits, and the rabbits had elevated antibodies against the various structural proteins of HTLV-I. Four rabbits inoculated with HTLV-I-infected autologous lymphoid cells intravenously (i.v.) and intracerebrally (i.c.) had virus present in their PBMNC for more than 40 weeks, while those that were inoculated either with HTLV-I-infected human lymphoid cells or with autologous rabbit lymphoid cells intraperitoneally (i.p.) had episodes during which virus was not recovered from their PBMNC. The one rabbit inoculated i.p. developed antibodies to viral envelope glycoproteins earlier than did those inoculated i.v. and i.c. Rabbit lymphoid cell lines persistently infected with HTLV-I were established by cocultivating the rabbit PBMNC with HTLV-I-infected human lymphoid cells that had been irradiated or by inoculation with cell-free supernatant fluids of HTLV-I infected non-irradiated lymphoid cell cultures. HTLV-I-infected rabbit cell lines were of T-cell origin and expressed HTLV-I antigens by immunofluorescence. Electron microscopy revealed type-C retrovirus particles.Type of Medium: Electronic ResourceURL: -
19Articles: DFG German National LicensesStaff View
ISSN: 1573-7284Keywords: Creutzfeldt-Jakob disease ; Tubulovesicular structuresSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Tubulovesicular structures (TVS) have been consistently observed in brain tissue of animals with transmissible spongiform encephalopathies such as natural and experimental scrapie, bovine spongiform encephalopathy, and experimental Creutzfeldt-Jakob disease (CJD). In this communication we demonstrate for the first time the presence of TVS in natural CJD. TVS were detected in all 3 CJD specimens. However, they were rare and were found only in one or two locations per grid. They were seen in distended pre- and postsynaptic terminals and measured approximately 35 nm in diameter, and they were smaller and of higher electron density than synaptic vesicles. Their occurrence in all types of spongiform encephalopathies irrespective of the affected host and the strain of infectious agent emphasizes their biological significance.Type of Medium: Electronic ResourceURL: -
20Articles: DFG German National LicensesLiberski, P. P. ; Kwiecinski, H. ; Barcikowska, M. ; Mirecka, B. ; Kulczycki, J. ; Kida, E. ; Brown, P. ; Gajdusek, D. C.
Springer
Published 1991Staff ViewISSN: 1573-7284Keywords: PrP-arnyloid ; Creutzfeldt-Jakob diseaseSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract We report here PrP-immunohistochemistry performed on brains from CJD cases from Poland. Only one of five definitive CJD cases exhibited typical PrP-immunoreactive kuru-like plaques and this was case of a short duration. We thus confirm the low frequency of PrP plaques in CJD of Eastern and Central European origin.Type of Medium: Electronic ResourceURL: