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1
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Prion Diseases: a dual view of the prion hypothesis as seen from a distance

100%
Liberski P. P., Jaskolski M.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
197-226
EN
We review the historical background and principles of the prion theory in its current shape. We showed that most of data may be still interpreted dually according to the protein only hypothesis and according to the theory in which additional component is necessary to comprise the infectivity. The enormous impact of structural biological studies is also stressed.
2
Content available remote

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.
3
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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.
Acta Neurobiologiae Experimentalis
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2002
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vol. 62
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issue 3
121-129
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.
4

Gelatine and prions - evaluation of consumers safety

80%
Fikus M.
Biotechnologia
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1998
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issue 3
9-13
EN
Gelatine is defined as a mixture of polypeptides obtained by partial non-enzymatic hydrolysis of collagen contained in bones and skins mainly bovines and/or pigs after the following treatments: degreasing, acid treatment, and/or alkaline treatment, washing, filtration, ion exchange and sterilization. Gelatine is produced for consumption and industrial purposes, and it is widely used as a component of various products. Scrapie and BSE are extremely resistant to various physical and chemical treatments. The gelatine can only be produced safely from healthy animals. European Community as well as FDA in the USA have paid close attention to this problem and issued special reports and rules on that matter.
5

80%
Piasecki E.
Postępy Higieny i Medycyny Doświadczalnej
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1993
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vol. 47
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issue 2
85-99
EN
are proteinaceous infection particles. They are probably devoid of nucleic acid. (PrP) is encoded by the normal cellular gene. of prion protein has different conformation than the normal PrP protein. The exact nature of the posttranslational modification of prion protein is unknown. This modification may be caused by infection with prions infectious disease or occures spontaneously sporadic disease. Some mutations in PrP gene results in the production of pathological protein familial disease.
6
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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.
7

Deletion/insertion polymorphism of the prion protein gene (PRNP) in Polish Holstein-Friesian cattle

80%
Czarnik U., Zabolewicz T., Strychalski J., Grzybowski G., Bogusz M., Walawski K.
Journal of Applied Genetics
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2007
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vol. 48
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issue 1
69-71
EN
The aim of the present study was to identify the deletion/insertion polymorphism of the bovine prion protein gene (PRNP) within the promoter sequence (23 bp), intron 1 (12 bp) and 3' untranslated region (14 bp). DNA was isolated from blood of 234 randomly tested Polish Holstein-Friesian cows and from semen of 47 sires used for artificial insemination (AI) in 2004. No statistically significant differences were found in the frequency of genotypes and alleles between cows and breeding bulls in the 3 analysed polymorphic sites within the PRNP gene. Only 3 haplotypes were identified in sires and 4 haplotypes in cows.
8

Deletion/insertion polymorphism of the prion protein gene (PRNP) in Polish Holstein-Friesian cattle

80%
Czarnik U., Zabolewicz T., Strychalski J., Grzybowski G., Bogusz M., Walawski K.
Journal of Applied Genetics
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2007
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vol. 48
|
issue 3
69-71
EN
The aim of the present study was to identify the deletion/insertion polymorphism of the bovine prion protein gene (PRNP) within the promoter sequence (23 bp), intron 1 (12 bp) and 3' untranslated region (14 bp). DNA was isolated from blood of 234 randomly tested Polish Holstein-Friesian cows and from semen of 47 sires used for artificial insemination (AI) in 2004. No statistically significant differences were found in the frequency of genotypes and alleles between cows and breeding bulls in the 3 analysed polymorphic sites within the PRNP gene. Only 3 haplotypes were identified in sires and 4 haplotypes in cows.
9

Prions - the new infectious agent

70%
Gogiel T.
Biotechnologia
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1998
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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.
10
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.
11

Presence and significance of the infectious elements in technologically relevant yeasts' cells

61%
Dzwonek M., Gniewosz M., Primik M., Duszkiewicz-Reinhard W.
Biotechnologia
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2005
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issue 3
202-208
EN
The killer phenotype of Saccharomyces cerevisiae strains is based upon the presence of viruses in the cytoplasm of the yeast cells. Resent analysis of the molecular basis in these phenomenon let researchers to reveal its molecular mechanism and ecological function. Mammalian transmissible spongiform encephalopathiesare is likely due to the propagation of an abnormal form of some protein. Such infectious agents, which are termed prions, exist in yeasts. This review highlights the variety of infectious elements present in Saccharomyces cerevisiae as well as their influence on the yeasts properties.
12
Content available remote

Molecular biology of brain aging and neurodegenerative disorders

61%
Wisniewski T., Frangione B.
Acta Neurobiologiae Experimentalis
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1996
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vol. 56
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issue 1
267-279
EN
A significant component of the aging process is genetically determined. Numerous theories of aging exist, many of which postulate the existence of "longevity genes". Recent advances in molecular biological and other techinques have allowed a significantly greater understanding of aging and age-related disease. This will be illustrated by four genetic and sporadic diseases: Alzheimer's disease (AD) and related disorders, transthyretin dementia, cerebral amyloid angiopathy-Icelandic type and scrapie related diseases. Alzheimer's disease (AD), the most common of this group, is the leading cause of dementia in Western countries. Recent genetic and biochemical studies have shown in involvement of at least four genes in the pathogenesis of AD. In early-onset familial AD mutations in the (beta)PP, S182 (presenilin 1) and STM2 (presenilin 2 or E5-1) genes have been found, while in the more common late-onset AD the presence of the apolipoprotein E4 isotype is a major risk factor. Genetic studies have also helped to elucidate the etiology of rarer cerebral amyloidoses such as the recently described Hungarian amyloidosis that is characterized by meningocerebrovascular amyloid deposition, with resultant dementia. This disease is linked to a mutation in the transthyretin gene. It is hoped that in the near future this increase in knowledge will allow the development of therapeutic approaches to slow the aging process.
13

Prion protein gene polymorphism in healthy and BSE-affected Slovak cattle

61%
Hresko S., Mojzis M., Tkacikova L.
Journal of Applied Genetics
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2009
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vol. 50
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issue 4
371-374
EN
Variation of the PrP gene was examined in healthy and BSE-affected Slovak cattle. According to previous studies, the 23-bp indel polymorphism is supposed to be associated with higher susceptibility to BSE. We investigated 301 samples from healthy cattle of various Slovak breeds and 24 samples obtained from tissues of BSE-affected cattle in Slovakia. We examined the PrP gene for the 23-bp indel polymorphism in the putative promoter region, 12-bp indel polymorphism in the first intron of the PrP gene, variations in number of octapeptide repeat units, and presence of the silent AAC?AAT transition in codon 192 within the protein-coding region of the PrP gene. Altogether we found 23 different genotypes in the group of healthy cattle and only 6 genotypes in the group of BSE-affected cattle. Comparison of homozygotes for the 23-bp insertion and heterozygotes showed significant differences (P < 0.05) in genotype distribution between the examined groups. Thereby the homozygous insertion genotype at the 23-bp indel polymorphism site in the promoter region of the prion protein gene seems to have a protective effect against BSE.
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