Publications

amyloidosis.	amyloidosis is a disease involving the fibrillar deposition of proteins in a manner that uniformly leads to the presence of green birefringence on polarization microscopy after staining the involved tissues with congo red. in the year summarized, a wide range of new information has accumulated about this disease. in this article, attention has been paid to several newly described proteins now known to precipitate into amyloid deposits, including the proteins transthyretin, apolipoprotein a-1, cy ...	1991	2043438
workshop on scrapie-related disease control.		1991	2065368
scrapie in sheep and goats.		1990	2075694
experimental scrapie in white swiss mice.	in order to confirm the clinical and histological diagnosis of scrapie and to determine the infectivity titer of the scrapie agent in the brain of a naturally infected suffolk sheep, 123 white swiss mice were inoculated intracerebrally. from about 13 to 20 months post-inoculation, 28 mice died, and 95 that were sick were killed. in the terminal stages of disease, the mice developed weakness, gradual emaciation, posterior ataxia, and occasionally alopecia. the average infection (83%) of mice affe ...	1990	2082631
cytoskeletal messenger rna stability in human neocortex: studies in normal aging and in alzheimer's disease.	total rna was extracted from human brain temporal and parietotemporal neocortical grey matter with postmortem intervals (pmi) of up to 13.5 hours. the integrity and rank abundance of heterogeneous nuclear rna (hnrna) and messenger rna (mrna) were analyzed by northern gel dot blot hybridization with specific cloned probes of neurobiological interest: the rna messages for four cytoskeletal components including glial fibrillary acidic protein (gfap), alpha-tubulin, beta-actin and the human neurofil ...	1990	2084053
[the biology of prions, a response to the enigma of spongiform encephalopathies of sheep and humans].	prions are small proteinaceous infectious particles without nucleic acids. the prion protein arise from a cellular protein by a post-transduction event and constitute amyloid deposits. prions are the agent of animal and human spongiform encephalopathies which evolve on simultaneous infectious and genetic dependence in the absence of a detectable immune response.	1990	2094563
mad cows and englishmen: bovine spongiform encephalopathy.		1990	2096311
[recent advances in the transmissible spongiform encephalopathy research--with special reference to scrapie].		1990	2098959
scrapie agent in muscle.		1990	2105554
vacuolization, incubation period and survival time analyses in three mouse genotypes injected stereotactically in three brain regions with the 22l scrapie strain.	in previous studies we showed that c57bl mice injected stereotactically in the cerebellum with the 22l scrapie strain had a significantly shorter incubation period than those injected with the same agent in other brain regions. in mice injected in the cerebellum, vacuolization was limited to the cerebellum, medulla and mesencephalon, whereas injection into forebrain regions resulted in vacuolization in all brain regions. the studies suggested that the cerebellum had a selective vulnerability for ...	1990	2106573
pathogenesis and pathology of scrapie after stereotactic injection of strain 22l in intact and bisected cerebella.	the mechanisms involved in the spread of scrapie within the brain remain unclear. to examine this issue the 22l scrapie strain was injected in one side of the cerebellum of mice in which the cerebellum had been bisected prior to injection. another group of animals received the same injection into intact cerebella, i.e. without prior bisection. we found that bisection of the cerebella delayed the spread of scrapie agent from the injection site to the contralateral side of the cerebellum and that ...	1990	2106574
resistance of scrapie infectivity to steam autoclaving after formaldehyde fixation and limited survival after ashing at 360 degrees c: practical and theoretical implications.	scrapie-infected hamster brains and their extracted amyloid fibrils were subjected to formaldehyde and steam autoclaving, alone or in combination. treatment with formaldehyde before autoclaving stabilized infectivity, whereas treatment after autoclaving was either inactive or further reduced infectivity. in additional experiments on specimens (not treated with formaldehyde) that were subjected to dry heat, a small amount of infectivity still survived a 1-h exposure to temperatures as high as 360 ...	1990	2107265
bovine spongiform encephalopathy.		1990	2107889
potential retroviral rnas in creutzfeldt-jakob disease.	the molecular nature of the related infectious agents that cause creutzfeldt-jakob disease (cjd) and scrapie is poorly understood, and an agent-specific nucleic acid genome has not yet been identified. several biological manifestations of these agents resemble those seen in retrovirus-induced diseases. we therefore attempted to identify an agent-specific retrovirus-like rna transcript in cjd infectious fractions. a series of synthetic oligonucleotides complementary to known mammalian retroviral ...	1990	2108258
further characterization of scrapie replication in pc12 cells.	the rat pheochromocytoma cell line, pc12, undergoes neuron-like morphological, biochemical and electrophysiological differentiation, in the presence of low concentrations of nerve growth factor (ngf). ngf-treated pc12 cells have been shown previously to support 139a scrapie agent replication. in the present report we extended these findings and analysed the cellular conditions necessary for agent replication. following the infection of differentiated pc12 cells, scrapie replicated to relatively ...	1990	2109041
scrapie and human neurodegenerative diseases.		1990	2109648
is bse simply scrapie in cattle?		1990	2111595
methods for inactivating experimentally induced scrapie in hamster tissues.		1990	2112123
conservation of infectivity in purified fibrillary extracts of scrapie-infected hamster brain after sequential enzymatic digestion or polyacrylamide gel electrophoresis.	infectious extracts of scrapie-infected hamster brain enriched for scrapie-associated fibrils and scrapie amyloid protein (prp) were partially denatured and subjected to either polyacrylamide gel electrophoresis with subsequent isolation of the prp band or sequential enzymatic digestion with deglycosidase, phospholipase, proteinase, and several different nucleases. infectivity measurements of these various specimens revealed a convincing association between infectivity and scrapie amyloid protei ...	1990	2119503
the search for scrapie agent nucleic acid.	despite decades of research, the identity of the scrapie agent has remained elusive. recent studies have discovered much about the influence of the host genome upon scrapie infection, yet relatively little is known about the causative agent itself. the predominant hypothesis in the scrapie field (the prion hypothesis) argues that the disease is the result of an infectious protein and that nucleic acid is not required for infection. biological studies of the scrapie agent, however, suggest that a ...	1990	2120561
incubation periods and histopathological changes in mice injected stereotaxically in different brain areas with the 87v scrapie strain.	after stereotaxic injection into five different brain areas (cortex, caudate nucleus, substantia nigra, thalamus and cerebellum) of im mice with the 87v scrapie strain, the cerebellum had the shortest incubation period. the vacuolation pattern was similar regardless of the area injected with extensive vacuolation in the thalamus, mesencephalon and hypothalamus. the pattern of amyloid plaques differed markedly depending on the area injected. in particular, no plaques were seen anywhere in the bra ...	1990	2122631
what causes scrapie?		1990	2123843
[concerning the so called prions].		1990	2130248
organ-specific modification of the dose-response relationship of scrapie infectivity.	the dose-response relationships of scrapie strain 263k-infected hamster brain and spleen homogenates were compared to determine if intracerebral end-point titrations of infectivity in these homogenates were measures of the same pathogenic phenomenon. analysis of the dose-response curves indicated that the average increase in incubation period per 10-fold dilution (i.e., the dilution kinetics) of brain infectivity was significantly different from that of spleen infectivity. this difference contra ...	1990	2138656
intraperitoneal infection with scrapie is established within minutes of injection and is non-specifically enhanced by a variety of different drugs.	single intraperitoneal (i.p.) doses of 16 different drugs were given to mice 2 h before injecting scrapie i.p. scrapie was injected as serial ten-fold dilutions of standard inocula and the effective titres obtained were used as a measure of the relative efficiency of infection in treated compared to saline injected mice. despite the wide variety of drugs tested, most of them increased, non-specifically, the efficiency of infection by 0.6 to 2.1 log10 i.p. ld50 units (i.e., 4 to 126-fold), but on ...	1990	2142415
pathogenesis of hamster scrapie. adherent splenocytes are associated with relatively high levels of infectivity.		1990	2143064
appearance of tubulovesicular structures in experimental creutzfeldt-jakob disease and scrapie precedes the onset of clinical disease.	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 ...	1990	2160182
[current developments in the problem of prions and prion diseases].	the present paper reports data referring to the mechanism of the cns degenerating process of prion origin, the role of the biological timing in prion diseases, the interrelations between the prion diseases and alzheimer's disease, new data on the normal cell protein prpc and prion protein in the prpsc scrapie, the physico-chemical properties of the prion prp-rn am properties the presence of the nucleic acid in the structure of prion, interrelations between prion and aids/visna retroviruses. the ...	1990	2173118
bovine spongiform encephalopathy and biological products for human use. report of an informal meeting held at nibsc on 16 may 1988.		1990	2180445
mad cows and the minister.		1990	2188132
proceedings of an international roundtable on bovine spongiform encephalopathy.		1990	2189852
scrapie in france.		1990	2189853
prion diseases of the central nervous system.	prions are novel, transmissible pathogens causing degenerative diseases in humans and animals. kuru, cjd, and gss illustrate the infectious, sporadic, and genetic mechanisms for human prion diseases, respectively. scrapie of sheep and goats is the prototypic prion disorder since it was the first of these diseases to be transmitted to laboratory rodents. over the past five years, a large amount of experimental data about the particles causing scrapie has been accumulated. most of the information ...	1990	2192281
survey of encephalopathy incidence set up by health ministry.		1990	2192493
oravske kuru. a human dementia raises the stakes in mad cow disease.		1990	2198656
multidisciplinary team dismisses bse 'threat'.		1990	2198689
bovine spongiform encephalopathy/scrapie.		1990	2201127
immunoreactive a4 and gamma-trace peptide colocalization in amyloidotic arteriolar lesions in brains of patients with alzheimer's disease.	cerebral amyloid angiopathy (caa) defines a biochemically heterogeneous entity that manifests as effacement of cerebral microvessel walls by a fibrillar material with characteristic tinctorial properties. in biochemical terms, the amyloid that infiltrates blood vessels in caa is composed of the a4 or beta peptide of alzheimer's disease (ad), a molecule related to gamma trace or cystatin c (seen in patients with hereditary cerebral hemorrhage with amyloidosis in iceland, hchwa-i), or the prp char ...	1990	2201197
[british cattle plague--also a danger for man? bovine spongiform encephalopathy (bse)--scrapie disease].		1990	2210580
scrapie: minor disease, potentially major problem.		1990	2211287
bovine spongiform encephalopathy--a new disease transmissable to humans?	current concerns about the cattle disease bovine spongiform encephalopathy do not appear to take cognisance of the parallel with scrapie, the similar/identical disease of sheep/goats. this has existed for 200 years, and clearly involved a longstanding consumption of meat/offal from affected animals, but apparently without consequential human disease. a summary is given of the characteristics of the human and animal spongiform encephalopathy diseases and their causative agents. the conclusion of ...	1990	2233424
unsolved problems confronting the sheep industry.	to support a sheep production system, veterinary practitioners must integrate diagnosis, treatment, and prevention with management, nutrition, and economics. scrapie, spider syndrome, prolapses, and parasitism are a few of the less dramatic but constant problems in sheep practice. problems with drug availability and residues may be as difficult to solve as predation and economic problems.	1990	2245376
detection of single-stranded dna in scrapie-infected brain by electron microscopy.	the nucleic acid content of enriched preparations of mitochondria/tubulofilamentous particles from normal and scrapie-infected hamster brains were examined by electron microscopy. after spreading on collodion-coated grids circular molecules of approximately 15.7 kb corresponding in size to mitochondrial dna (mtdna) were observed both in normal and scrapie-infected brains. in nucleic acid preparations from scrapie-infected brains multimeric mtdna and single-stranded dna strands of about 0.49 x 10 ...	1990	2258920
a questionnaire survey of the prevalence of scrapie in sheep in britain.	an anonymous, self-administered questionnaire has been used in two independent surveys to try to determine the prevalence of scrapie in the national sheep flock. the disease was recorded in 35 counties in england and wales. about a third (26.5 and 37.3 percent) of respondents owning 100 or more sheep indicated that they had seen sheep with scrapie in their flocks. the incidences of clinical cases recorded in affected flocks in the two surveys were 0.5 and 1.1 cases/100 ewes/year. at present ther ...	1990	2260251
the use of monosodium glutamate in identifying neuronal populations in mice infected with scrapie.	the excitatory amino-acid, monosodium glutamate, which causes degeneration in the retinal ganglion cells in neonatal mice, was used to investigate the transport of scrapie within optic nerve axons. in treated mice, there was prolongation of the incubation period following intraocular infection with the me7 strain of scrapie, and a decrease in the severity of retinopathy after intracerebral infection with the 79a strain. these data confirm that scrapie infection spreads along neural pathways, and ...	1990	2263317
spongiform encephalopathy in an arabian oryx (oryx leucoryx) and a greater kudu (tragelaphus strepsiceros)	clinical, pathological and epidemiological details of scrapie-like encephalopathies are described in an arabian oryx and a greater kudu. clinical signs included ataxia and loss of condition with a short, progressive clinical course (22 and three days, respectively). histopathological examination of the brains revealed spongiform encephalopathy characteristic of that observed in scrapie and bovine spongiform encephalopathy (bse). it seems probable that these cases have a common aetiology with bse ...	1990	2264242
prevalence of scrapie in british flocks.		1990	2267716
[bovine spongiform encephalopathy: a new entity caused by a non-conventional transmissible agent].	in 1986, a new neurologic disease appeared in the great britain's cattle. according to its histological lesions, this condition belongs to the group of transmissible encephalopathies known as spongiforme encephalopathies (se). these se are associated with no-conventional transmission agent (ncta) or prion. at the time of writing, over 13,000 cases of bovine spongiforme encephalopathy (bse) have occurred in uk. the most likely origin of this dramatic outbreak would be an oral contamination of cat ...	1990	2271982
bovine spongiform encephalopathy in the united states.		1990	2278551
pancreatic lesions and hypoglycemia-hyperinsulinemia in scrapie-injected hamsters.	hamsters injected intracerebrally with scrapie strains 139h or 22ch or with normal hamster brain were assessed for body weight periodically throughout the incubation period. animals injected with the scrapie strains became obese before the appearance of the motor changes that are indicative of the start of clinical disease. during the latter part of the incubation period and during clinical disease, animals were hypoglycemic and showed marked hyperinsulinemia. at autopsy, there was marked hyperp ...	1990	2313125
bse, scrapie and laboratory models.		1990	2316164
transmission of encephalopathies.		1990	2321340
tainted feed, mad cows. could a british cattle disease infect u.s. herds?		1990	2333492
library subtraction of in vitro cdna libraries to identify differentially expressed genes in scrapie infection.	we have developed a system where double-stranded cdna can be amplified using a synthetic oligonucleotide primer and the polymerase chain reaction, generating cdna libraries in vitro. using a library subtraction strategy (1), scrapie and control brain in vitro cdna libraries were used to identify sequences whose expression is modulated in scrapie infection. one of these sequences represents beta-2 microglobulin, while the other two have not been previously described. the use of in vitro libraries ...	1990	2339063
changes in the distribution of anionic sites in brain micro-blood vessels with and without amyloid deposits in scrapie-infected mice.	cationic colloidal gold (ccg) and scrapie-infected mouse brain samples embedded in lowicryl k4m were used for ultrastructural localization of negatively charged microdomains (anionic sites) in the cerebral microvasculature. the distribution of anionic sites on both fronts (luminal and abluminal) of endothelial cells and in the basement membrane (bm) in the majority of micro-blood vessels (mbvs) located outside the plaque area and in the remaining cerebral cortex was similar to that which has bee ...	1990	2339589
new fears on transmission.		1990	2342557
an overview of scrapie in the united states.		1990	2347765
experimentally induced bovine spongiform encephalopathy in cattle in mission, tex, and the control of scrapie.		1990	2347766
the scrapie control program in the united states.		1990	2347767
recommendations for initiation of research studies on bovine spongiform encephalopathy.		1990	2347769
histopathology and image analysis of brain lesions in ovine scrapie in japan.		1990	2348610
ministerial madness avoided.		1990	2348868
the unexpected export?		1990	2348869
frankness needed.		1990	2359438
link between scrapie and bse?		1990	2366871
spongiform encephalopathy in an eland.		1990	2368268
mps to look at government handling of bse.		1990	2368285
bse risks.		1990	2368290
coexistence of creutzfeldt-jakob disease and alzheimer's disease in the same patient.	we report the case of a 73-year-old patient in whom a diagnosis of creutzfeldt-jakob disease, suggested by the clinical course, was verified by the neuropathologic finding of widespread spongiform change and astrogliosis, the presence of proteinase-resistant protein in brain extracts, and the experimental transmission of spongiform encephalopathy to primates inoculated with brain tissue. however, neuropathologic examination also revealed a profusion of senile and neuritic plaques and neurofibril ...	1990	2405293
three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins.	given the critical role of the prion protein (prp) in the transmission and pathogenesis of experimental scrapie, we investigated the prp gene and its protein products in three hamster species, chinese (cha), armenian (aha), and syrian (sha), each of which were found to have distinctive scrapie incubation times. passaging studies demonstrated that the host species, and not the source of scrapie prions, determined the incubation time for each species, and histochemical studies of hamsters with cli ...	1990	2406562
transmissible encephalopathies in animals.	scrapie in sheep and goats is the best known of the transmissible encephalopathies of animals. the combination of maternal transmission of infection and long incubation periods effectively maintains the infection in flocks. a single sheep gene (sip) controls both experimental and natural scrapie and the discovery of allelic markers could enable the use of sire selection in the control of the natural disease. studies of experimental rodent scrapie show that neuroinvasion occurs by spread of infec ...	1990	2407328
the molecular mechanisms of scrapie encephalopathy and relevance to human neurodegenerative disease.	we have investigated alterations in the structure and function of nuclei isolated from normal and pathological brains in a number of neurodegenerative diseases including scrapie and alzheimer's disease. here we summarize both general and specific changes in chromatin structure, gene expression, and neuropathological features for each encephalopathy and compare them in terms of their molecular biological similarities and differences. while both scrapie and alzheimer's disease share a number of co ...	1990	2407330
[encephalopathy caused by prions].	the concept of prion encephalopathy has emerged from such previous notions as slow virus infections, spongiform encephalopathies or transmissible dementias. the term prion (prusiner, 1982) is now used in preference to unconventional agents. proteins and genes of prions have recently been identified by molecular biology. exactly how prion proteins are amplified in cells is still unknown. it has been demonstrated that amyloid deposits in scrapie-infected brain, creutzfeldt-jakob, gerstmann-straüss ...	1990	2408126
specific proteins associated with creutzfeldt-jakob disease and scrapie share antigenic and carbohydrate determinants.	small amounts of brain tissue (2 g) infected with creutzfeldt-jakob disease (cjd) can be fractionated by using a simple 1-day method that includes lysis with n-lauroylsarcosine. unique fibrils have been identified previously in scrapie- and cjd-infected tissue. these fibrils were abundant in final fractions. preparations from human cjd autopsy material and from experimental hamster and guinea pig cjd all displayed readily identifiable fibrils that were not seen in control preparations. thus, the ...	1985	2408277
characterization of nucleic acids in membrane vesicles from scrapie-infected hamster brain.	this study reports the partial characterization of nucleic acids present in gradient fractions enriched for large membrane vesicles from scrapie-infected and uninfected hamster brains. labeling of phenol-extracted nucleic acids at the 3' or 5' ends revealed abundant amounts of low-molecular-weight rna and little or no dna. these nucleic acids survived nuclease treatment of membrane vesicles but were sensitive to rnase after phenol extraction. analysis of 5'-end-labeled nucleic acids by one- and ...	1985	2409296
neurotransmitter metabolites, enzymes and receptors in experimental scrapie.	a comprehensive study has been made of metabolites, enzymes and receptors for a variety of neurotransmitter systems in different parts of the cns from mice affected with 139a scrapie. studies were made at the early clinical stage so as to minimise secondary alterations in terminally sick mice. even though histological lesions (vacuolation) are widespread in the cns in this model of scrapie, no consistent neurochemical changes were found in mid- or anterior brain. in the cerebellum, the activity ...	1985	2414405
antibody to scrapie-associated fibril protein identifies a cellular antigen.	antibody to scrapie-associated fibril (saf) protein was produced in a rabbit by a primary injection of 50 brain equivalents of native saf protein emulsified in complete freund's adjuvant and by a secondary inoculation of polyacrylamide gel which contained 50 brain equivalents of saf protein emulsified with incomplete freund's adjuvant. the antibody produced was found to decorate the saf as demonstrated by the use of goat anti-rabbit colloidal gold second antibody. immunoblots (dot blots and west ...	1986	2418150
the pathogenesis of slow virus infections: molecular analyses.	slow infections raise some novel issues in understanding the pathogenesis of viral diseases that have been increasingly addressed at the molecular level with in situ hybridization. these developments are reviewed as the framework for discussion of the realized and potential impact of these investigations for major neurological afflictions of humans and for aids.	1986	2419455
prolongation of scrapie incubation period by an injection of dextran sulphate 500 within the month before or after infection.	a single intraperitoneal injection of 250 micrograms dextran sulphate 500 (ds500) reduced the susceptibility of mice to scrapie given by the same route. a lower dose (25 micrograms) was less effective but still produced significant incubation period lengthening, while a high dose (2.5 mg) further increased the degree of prolongation. this reduced susceptibility occurred with ds500 administered up to at least 4 weeks prior to intraperitoneal scrapie inoculation and up to at least 2 weeks after sc ...	1986	2419489
molecular hybridization studies with scrapie brain nucleic acids. ii. differential expression in scrapie hamster brain.	rna and dna fractions prepared from nucleic acids obtained from normal and scrapie-infected hamster brains following exhaustive treatment with nuclease were used as probes in a search for a specific scrapie nucleic acid. employing rna blot hybridization a 2.3 kb rna was detected in higher concentration in scrapie-infected hamster brains than in normal controls. our data suggests that scrapie disease may be associated with the differential expression of a series of host genes.	1986	2420312
detection of scrapie-associated fibril (saf) proteins using anti-saf antibody in non-purified tissue preparations.	antisera raised to scrapie-associated fibril (saf) proteins were used to detect scrapie-specific polypeptides in three different non-purified brain preparations: a synaptosomal-mitochondrial fraction, 20% brain homogenate and 20% brain homogenate extracted with sarkosyl. the concentration of saf proteins in the preparations was greater than the quantity of saf as detected by negative stain electron microscopy. this suggests that not all of the protein exists in the form of saf. an immunologicall ...	1986	2420924
a 54-kda normal cellular protein may be the precursor of the scrapie agent protease-resistant protein.	scrapie is the best understood of the transmissible spongiform encephalopathies. these neurologic disorders include the human diseases kuru and creutzfeldt-jakob disease and are caused by pathogens with unique biological and molecular properties. one major protein, protease-resistant protein (prp)-27-30, is present in fractions isolated from scrapie-infected hamster brain that contain highly purified scrapie agent. prp-27-30 appears to be the major protein component of the hamster scrapie agent. ...	1986	2421288
changes in the serotonergic, noradrenergic and dopaminergic levels in the brain of scrapie-infected rats.	levels of serotonin (5-ht), 5-hydroxyindoleacetic acid (5-hiaa), dihydroxyphenylacetic acid (dopac) and 3,4-dihydroxyphenylethyleneglycol (dhpg) were determined by high-performance liquid chromatography in different brain areas of scrapie-infected rats, 8.5 months after intracerebral inoculation of a rat-adapted strain from mice brain (c 506). at this time, rats developed early clinical signs of the disease. scrapie-infected rats showed a reduction in the levels of 5-ht and 5-hiaa (frontal corte ...	1986	2421836
partial copurification of scrapie-associated fibrils and scrapie infectivity.	the association between scrapie infectivity and scrapie-associated fibrils (saf) during a partial purification procedure for infectivity was investigated. scrapie infectivity and saf can be separated from most membrane components by subcellular fractionation of infected mouse brain to obtain a synaptosomal fraction, followed by detergent treatment and density gradient centrifugation. after different detergent treatments, with either octyl glucoside or sodium n-lauroyl sarcosinate, saf showed dif ...	1986	2422138
scrapie and cellular prion proteins share polypeptide epitopes.	purified preparations of scrapie prions contain one major protein, prp 27-30, and aggregates of rod-shaped structures. on the basis of the nh2-terminal amino acid sequence of prp 27-30, a synthetic peptide (prp-p1) was constructed. monospecific rabbit antisera to prp-p1 were found by immunoblotting to react with prp 27-30 and its precursor (prp 33-35sc), as well as with a related protease-sensitive cellular homologue (prp 33-35c). an enzyme-linked immunosorbent assay showed that rabbit antiserum ...	1986	2422294
immunological comparison of scrapie-associated fibrils isolated from animals infected with four different scrapie strains.	scrapie-associated fibrils (safs) are abnormal filamentous structures that are uniquely associated with unconventional slow virus diseases. the antigenic relationships of safs from animals infected with four biologically distinct scrapie strains were investigated by using antisera raised to purified saf proteins. rabbit antisera were raised to safs isolated from mice infected with the me7 scrapie strain and to safs isolated from hamsters infected with the 263k scrapie strain. a strong antigenic ...	1986	2426470
suppression of scrapie infection in mice by heteropolyanion 23, dextran sulfate, and some other polyanions.	studies of polyanions that suppress scrapie have been done to pinpoint the cell types in the lymphoreticular system which are important in pathogenesis and to suggest possible prophylactic or therapeutic strategies for the unconventional slow viruses. a regime of three daily injections of the inorganic heteropolyanion hpa-23 reduced the effective scrapie dose by more than 99%; i.e., some mice survived peripherally injected doses of 100 50% lethal dose units. the effect was greatest when the firs ...	1986	2430521
amphotericin b delays the incubation period of scrapie in intracerebrally inoculated hamsters.	the scrapie-infected hamster has been considered an excellent model for the study of slow virus diseases of man (creutzfeldt-jakob disease) and animals. at the moment no therapy is available for the cure of these fatal central nervous system diseases, although several drugs have been tested. we found that amphotericin b (amb), a polyene antibiotic, increased the incubation time of scrapie disease in animals infected by either the intraperitoneal or intracerebral route. hamsters inoculated with a ...	1987	2433387
is scrapie prp 27-30 related to aids virus?		1987	2433599
impairment of the cortical and thalamic electrical activity in scrapie-infected rats.	cortical and thalamic eeg and somatosensory evoked potential (sep) induced by stimulation of the somesthetic radiations were studied in scrapie-infected rats. animals were inoculated intracerebrally with a rat-adapted strain (originating in the c506 m3 mouse scrapie strain). eeg and sep were recorded from 9 to 17 months after inoculation (ti). abnormalities (paroxysmal bursts, isolated spikes) first occurred in the cortex (parietal areas) and later in the thalamus, where they were usually less m ...	1987	2434315
formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids.	the purpose of this study was to design a method by which immunoperoxidase staining can be applied to formalin-fixed, paraffin-embedded tissue sections to demonstrate amyloid deposits in cerebral and systemic amyloidotic tissues. we used anti-prion protein, anti-beta-protein, anti-amyloid a, and anti-prealbumin antisera. the tissue sections were first treated with 100% formic acid for 5, 20, or 60 minutes and the unlabeled immunoperoxidase method (biotin-streptavidin system reagents) was used. t ...	1987	2441141
developmental expression and regional distribution of the scrapie-associated protein mrna in the rat central nervous system.	the scrapie associated protein (sap) has been shown to be a normal brain protein of yet undefined function. this study demonstrates that rat brain sap mrna levels undergo a transcriptionally dependent increase during normal development. the interregional variation in the adult rat central nervous system (cns) is roughly 10 fold, with highest levels in the basal ganglion/thalamus and lowest levels in the spinal cord.	1987	2443218
scrapie prion protein contains a phosphatidylinositol glycolipid.	the scrapie (prpsc) and cellular (prpc) prion proteins are encoded by the same gene, and their different properties are thought to arise from posttranslational modifications. we have found a phosphatidylinositol glycolipid on both prpc and prp 27-30 (derived from prpsc by limited proteolysis at the amino terminus). ethanolamine, myo-inositol, phosphate, and stearic acid were identified as glycolipid components of gel-purified prp 27-30. prp 27-30 contains 2.8 moles of ethanolamine per mole. incu ...	1987	2444340
mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins.	antibody response in mice to scrapie-associated fibril proteins (protease-resistant proteins [prps]) was generated to different epitopes depending on the source of antigen. mice responded differently to prps isolated from scrapie-infected animals of homologous (mouse) versus heterologous (hamster) species. an enzyme-linked immunosorbent assay established to monitor this antibody response in mice immunized with prps was unable to detect such a response in scrapie-infected mice. a monoclonal antib ...	1987	2446004
autoclaving does not decontaminate formol-fixed scrapie tissues.		1988	2455203
isolation of cdnas of scrapie-modulated rnas by subtractive hybridization of a cdna library.	we have developed a subtractive cloning procedure based on the hybridization of single-stranded cdna libraries constructed in pi h3m, a vector containing the phage m13 origin of replication. we have used this strategy to isolate three transcripts whose abundance is increased in scrapie-infected brain. dna sequence analysis showed that they represent glial fibrillary acidic protein, metallothionein ii, and the b chain of alpha-crystallin; the latter two may represent a response to stress.	1988	2456582
unraveling prion diseases through molecular genetics.	prions are transmissible pathogens that cause degenerative diseases in humans and animals. unique attributes of prion diseases include infectious, sporadic and genetic manifestations, as well as progression to death, all in the absence of a detectable immune response. prions are resistant to chemical procedures that modify or destroy nucleic acids and are composed largely of a protein, designated prpsc. molecular cloning of a cognate cdna established a cellular host origin for prpsc protein and ...	1989	2473558
scrapie agent decontamination: implications for bovine spongiform encephalopathy.	bovine spongiform encephalopathy probably results from the use of commercial diets containing scrapie-contaminated ingredients. of all the chemical and physical decontamination procedures which are effective against conventional viruses, only high temperature autoclaving, high concentrations of sodium hypochlorite, and possibly molar sodium hydroxide, are useful against the group of unconventional transmissible agents which includes scrapie. the implications of this problem for the rendering ind ...	1989	2499090
the role of the spleen in the neuroinvasion of scrapie in mice.	the pathogenesis of 139a scrapie has been studied in cw mice infected intraperitoneally (i.p.), intravenously (i.v.) or subcutaneously (s.c.). in mice splenectomised before i.p. infection, the evidence points to a neuroinvasive pathway from visceral lymph nodes (and other sites of scrapie replication in the peritoneum) to the thoracic spinal cord. however, in non-splenectomised mice, the major neuroinvasive pathway is clearly from spleen to thoracic cord because i.p. incubation periods are short ...	1989	2499134
pathogenesis of scrapie in mice after intragastric infection.	infection via the gastrointestinal tract is likely to be a natural route of scrapie infection in sheep. this paper describes the pathogenesis of the 139a strain of scrapie introduced intragastrically (i.g.) into cw mice. there was an almost immediate uptake of infectivity and onset of replication in peyer's patches which preceded replication in spleen. splenectomy had no effect on incubation period suggesting that, in contrast to the intraperitoneal route, the spleen plays little or no role in t ...	1989	2499135