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1
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The transmissible brain amyloidoses: a comparison with the non transmissible brain amyloidoses of Alzheimer type

100%
Liberski P. P.
Acta Neurobiologiae Experimentalis
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1993
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vol. 53
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issue 1
337-349
EN
I report here the basic concepts of the transmissible and non-transmissible cerebral amyloidoses. The pathogenesis of both types of brain amyloidoses consist of a synthesis of amyloid precursor followed by its processing to yield a final postranslationally modified deposit. PrPc and APP serve as precursor proteins while modified PrP (PrPsc and PrP 27-30) and beta-A4 as final deposits in transmissible and non-transmissible brain amyloidoses, respectively. The hallmark of both types of brain amyloidoses is amyloid deposits immunureactive to appropriate amyloids. Other, mostly unspecific, neuropathological phenomena as neurofibrillary tangles, dystrophic neurites and granulovacuolar degeneration are also discussed. The only disease-specific structure for transmissible cerebral amyloidoses are tubulovesicular structures of unknown biological significance. They may even be the infectious virus.
2
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The tubulovesicular structures - the ultrastructural hallmark for all prion diseases

80%
Liberski P. P.
Acta Neurobiologiae Experimentalis
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2008
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vol. 68
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issue 1
113-121
EN
Tubulovesicular structures (particles ? TVS) are the only ultrastructural marker for all prion diseases as seen by thin-section electron microscopy as opposed to ?negative-staining' techniques. TVS are spheres or short rods of approximately 27 nm in diameter. That size of TVS is also the size of filter cut-off of infectivity as judged from the ultrafiltration studies and the size of the smallest infectious unit as recently estimated. TVS have been found in all naturally occurring and experimentally induced prion diseases, including variant Creutzfeldt-Jakob disease and human familial TSEs ? fatal familial insomnia and Gerstmann-Str?ussler-Scheinker disease. In longitudinal studies, the number of neuronal processes containing TVS correlates roughly with the incubation period and with infectivity. Hence, they are readily found in hamsters infected with the 263K strain of scrapie but it is very difficult to find them in human TSEs where titer is lower. The composition of TVS is unknown but they are not composed of PrP. Their consistent presence in all TSEs suggests the unexplained role at least of TSE pathogenesis.
3
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Codon 219 in Creutzfeldt-Jakob disease in Poland

80%
Bratosiewicz-Wasik J., Wasik T. J., Liberski P. P.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
149-151
EN
Prion diseases are a group of etiologically heterogenous diseases. In addition to familial cases linked to mutations of PRNP open reading frame they include also cases of unknown etiology. One of the susceptibility factors to sporadic as well as iatrogenic prion diseases are PRNP polymorphisms. In the present study, we analyzed sequences of the PRNP gene codon 219 of 16 Polish CJD cases and we found heterozygous GAG to GAT changes on the sense strand and only wild type sequence on an antisense strand. The RFLP technique was used to verify this divergence and only wild type sequences were revealed.
4

Importance of genetic factors in pathogenesis of subacute spongiform viral encephalopathies

80%
Wierzba W., Wajgt A.
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vol. 49
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issue 2
253-261
5
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Codon 129 polymorphism of the PRNP gene in normal Polish population and in Creutzfeldt-Jakob disease, and? the search for new mutations in PRNP gene?

80%
Bratosiewicz J., Liberski P. P., Kulczycki J., Kordek R.
Acta Neurobiologiae Experimentalis
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2001
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vol. 61
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issue 3
151-156
EN
Polymorphism at codon 129 of the prion protein gene (PRNP) is implicated both in susceptibility and phenotype of human prion diseases. We characterized the valine and methionine allele frequency at codon 129 in 109 individuals representing the normal Polish population and in 15 Polish CJD cases. The distribution of the genotype was 45% Met/Met, 39% Met/Val, and 16% Val/Val in the control group whereas, of the CJD cases, 73.3% were homozygous for methionine, 13.3% homozygous for valine and 13.3% were heterozygous. The novel missense mutation (ATGACG) at codon 232 was identified in one of the samples with a GSS phenotype.
6
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Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob disease in hamsters. I. Alterations of myelinated axons

80%
Liberski P. P., Bratosiewicz-Wasik J., Gajdusek D. C., Brown P.
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EN
Classical and ultrastructural neuropathology of prion diseases are generally well described. Here we report that alterations of myelinated fibres in hamsters infected either with polioencephalopathic strains of scrapie or panencephalopathic strains of CJD (Echigo-1) are virtually identical and differ only quantitatively. In contrast, mice infected with the panencephalopathic Fujisaki strain of CJD exhibited much more elaborate changes of myelinated fibres.
7
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Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob disease in hamsters. II. Astrocytic and macrophage reaction toward the axonal destruction

80%
Liberski P. P., Bratosiewicz-Wasik J., Gajdusek D. C., Brown P.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
131-139
EN
We report here the microglial (macrophage) and astrocytic reaction in several models of transmissible spongiform encephalopathies (TSEs) or prion diseases. With the low power electron microscopy it was readily apparent that myelinated vacuoles were surrounded by cells and their processes. The latter belonged either to hyperplastic reactive astrocytes or to macrophages. Typically reactive astrocytes exhibited cytoplasm filled with innumerable glial filaments and, occasionally, other organelles (like cilia) and abundant tortuous intercellular junctions of adhesive plaque junction type. Desmosome-like junctions connecting astrocytic elements were also seen. As described earlier, astrocytic processes were occasionally interdigitated with oligodendroglial cells and their processes. Two types of macrophages were readily described. The majority of them exhibited electron-dense cytoplasm and numerous ?empty? vacuoles (digestive chambers) containing cellular debris. Occasional vacuoles were surrounded by a thin collar reminiscent of ?lyre-like inclusions? of the second type of macrophages. The latter were rare and characterized by numerous ?lyre-like? inclusions. Several mylinated fibres were clearly engulfed by the cytoplasm of a macrophage containing unusual annulate lamellae.
8
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Proliferation of mesaxons in the optic nerve of hamsters infected with the Echigo-1 strain of Creutzfeldt-Jakob disease

80%
Walis A., Liberski P. P.
Acta Neurobiologiae Experimentalis
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1999
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vol. 59
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issue 3
177-180
EN
The Echigo-1 strain of CJD was isolated by Mori and colleagues from a case of 33-years-old female with a panencephalopathic type of CJD. An incubation period following intracerebral inoculation of hamsters with 10 % cleared suspension of the Echigo -1-affected brain was approximately six months. We report here ultrastructural changes in the optic nerves. Vacuoles developed within myelinated axons: within axoplasm or within the myelin sheath and these were accompanied by exuberant reaction of macrophages and hypertrophied astrocytes. Axons underwent Wallerian degeneration and dystrophic neurites were also seen. Most important, we observed proliferation of inner mesaxons. Cross-sectional profiles of innumerable myelinated fibers contained membranous organelles which were continuous with the inner lamellae of the oligodendroglial cells. These unusual proliferations of inner mesaxons formed whorls and elaborated loops. In some axons, proliferation was so severe that loops of mesaxon filled the whole cross-section of the axon. Occasionally, we observed intrusion of the membranous tongue of the inner mesaxon into axoplasm.
9
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Variant Creutzfeldt-Jakob Disease

80%
Will G. R.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
167-173
EN
Variant Creutzfeldt-Jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. The clinical and investigative features of variant CJD are relatively distinct from sporadic CJD. The number of cases of vCJD are increasing with time in the UK, but the total future number of cases of vCJD is uncertain.
10
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Tubulovesicular structures are present in brains of hamsters infected with the Echigo-1 strain of Creutzfeldt-Jakob disease agent

80%
Liberski P. P.
Acta Neurobiologiae Experimentalis
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2008
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vol. 68
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issue 1
39-42
EN
Tubulovesicular structures (particles; TVS) are virion-like particles 25?30 nm in diameter found by thin-section electron microscopy in brains of all prion diseases including scrapie, Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI) and Gerstmann-Str?ussler-Scheineker disease (GSS), as well as in cell cultures infected with TSE agents. TVS are regarded as a disease-specific ultrastructural marker for TSEs and, by those not completely satisfied with the prion hypothesis, they are even considered to be a possible candidate for the infectious TSE agent itself. A caveat regarding that interpretation stemmed from previous failures to find TVS by electron microscopic studies of tissues from animals infected with the Echigo-1 strain of CJD agent. We now report detecting TVS in brains of hamsters infected with that strain of CJD agent, albeit with a very low frequency.
11
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Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob disease

80%
Liberski P. P., Sikorska B., Bratosiewicz-Wasik J., Walis A., Brown P., Brown D.
Acta Neurobiologiae Experimentalis
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2003
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vol. 63
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issue 4
309-318
EN
We report here on the exuberant glial reaction in the optic nerves affected by prion diseases. Optic nerves from CJD- and GSS-, and scrapie-infected mice and hamsters showed severe pathology. These lesions were qualitatively indistinguishable from each other but were more intense in the Fujisaki model than in the hamsters inoculated with Echigo-1. Exuberant cellular reaction comprised of macrophages containing numerous mitochondria, abundant rough endoplasmic reticulum, and secondary lysosomes filled with digested myelin debris, electron-dense material and occasionally, entire myelin-bound vacuoles were readily observed in both models. Macrophages actively digesting myelin fragments and containing lyre-like bodies and paracrystalline inclusions were frequently noted. Some macrophages extended long filopodia to form labyrinth-like structures, and within a few macrophages, concentric arrays of cisterns and channels sequestrated part of the cytoplasm. An analogous network of narrow cisterns was seen to surround whole segments of the myelinated fibers.
12
Content available remote

Neuropathology of variant Creutzfeldt-Jakob disease

70%
Ironside J. W., Head M. W., McCardle L., Knight R.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
175-182
EN
The clinical, neuropathological genetic and biochemical features of variant Creutzfeldt-Jakob disease (vCJD) are compared to the 926 other cases of suspected CJD referred to the National CJD Surveillance Unit laboratory from 1990-2001. Histological studies of the central nervous system, lymphoid tissues and other organs were accompanied by immunocytochemistry for prion protein (PrP); Western blot analysis of PrPRES was performed on frozen brain tissue. The pathology of vCJD showed relatively uniform morphological and immunocytochemical characteristics, with PrP accumulation in lymphoid tissues, but not in other non-neural tissues. PrPRES accumulation in vCJD showed a uniform glycotype pattern distinct from sporadic CJD. All cases of vCJD were methionine homozygotes at codon 129 of the PrP gene. In view of the spread of bovine spongiform encephalopathy in Europe and Japan, continuing surveillance is required for all forms of CJD, with histological and biochemical analysis of suspected cases to allow an accurate laboratory diagnosis.
13
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Apoptosis in relation to neuronal loss in experimental Creutzfeldt-Jakob disease in mice

70%
Jesionek-Kupnicka D., Kordek R., Buczynski J., Liberski P. P.
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EN
Apoptosis constitutes a genetically determined process to eliminate superfluous or damaged cells in tissues. Deficiencies in apoptosis regulation are involved in different pathologies including prion diseases. Some experimental studies show that neuronal loss - one of the hallmarks of prion diseases may be accomplished by apoptosis. We evaluated twenty five mice infected experimentally with the Fujisaki strains of CJD and sacrified sequentially in one week intervals. Apoptotic cells in various brain regions were detected by in situ end labelling (TUNEL) and electron microscopy in comparison with neuronal cell loss. The number of labelled cells per brain was very low - from a few labelled cells 6 weeks after inoculation to a maximum of 14 in the terminal stage. The number of neurones counted in 8 selected areas were considerably lower in terminally sick animals (20 and 21 week of incubation period) than in control mice. The mean value of loss of neuronal cells was 32%. The greatest loss (55%) of neurones was noted in the septal nuclei of the paraterminal body and the least lost (16%) in the hypothalamus. Compared to the extensive neuronal loss (30-50%), the number of apoptotic cells detected by in situ end labelling seems to be very low, and the process of neuronal death become more intensive during the progression of the disease.
14

Prions - the new infectious agent

70%
Gogiel T.
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issue 3
35-51
EN
Prions are devoid of nucleic acids and they are composed mainly or exclusively of protein PrPSC, that is a conformational variant of the normal cellular prion protein PrPC, encoded by a chromosomal gene. Conversion of PrPC into PrPSC is a posttranslational process which is accompanied by the acquisition of high b-sheet content. Human prion diseases may be of sporadic, genetic or infectious origin. Human activity caused a 'mad cow disease' epidemic, iatrogenic Creutzfeldt-Jakob disease (CJD), and lately, a new variant of CJD, which is thought to be a result of transmission of bovine prions to humans. Prion diseases are always fatal, and there is a need to develop effective methods of prevention and therapy for these disorders.
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