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prion disease

Microglia Are Critical in Host Defense against Prion Disease

Microglia Are Critical in Host Defense against Prion Disease

... Ablation of microglia by PLX5622 treatment alters proinflammatory gene expression. Much of the inflammatory response associated with prion disease is proposed to be of microglial origin (30). Therefore, ...

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Mechanistic Insights into the Cure of Prion Disease by Novel Antiprion Compounds

Mechanistic Insights into the Cure of Prion Disease by Novel Antiprion Compounds

... of prion diseases and other amyloidoses. In prion diseases, Congo red and some analogues and derivatives have been shown to be effective in a variety of models, includ- ing a cell-free polymerization assay ...

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Accelerated Prion Disease in the Absence of Interleukin-10

Accelerated Prion Disease in the Absence of Interleukin-10

... to prion disease pathology as this cytokine can in- duce profound fibrosis ...terminal prion disease in IL-10 ⫺/⫺ mice implies that IL-10 may be expected to play an important role in limiting ...

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Chronic Subclinical Prion Disease Induced by Low-Dose Inoculum

Chronic Subclinical Prion Disease Induced by Low-Dose Inoculum

... mouse prion disease with clearly evident, progres- sive clinical signs, as described in Table ...1. Prion disease in- duced by ME7 or RML normally began with similar early ...of prion ...

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Oral Prion Disease Pathogenesis Is Impeded in the Specific Absence of CXCR5-Expressing Dendritic Cells

Oral Prion Disease Pathogenesis Is Impeded in the Specific Absence of CXCR5-Expressing Dendritic Cells

... single prion- dosed food pellet was then placed in the ...the prion-contaminated food ...After prion exposure, mice were coded and assessed weekly for signs of clinical disease and culled at a ...

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Effect of Microglial Inflammation in Prion Disease

Effect of Microglial Inflammation in Prion Disease

... neurodegenerative disease including Alzheimer’s disease, Parkinson’s disease, multiple sclerosis, Huntington’s disease, amyotrophic lateral sclerosis (ALS), and prion disease ...

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Pharmacokinetics of quinacrine in the treatment of prion disease

Pharmacokinetics of quinacrine in the treatment of prion disease

... Conclusions: The documented extensive brain tissue penetration is encouraging suggesting quinacrine might be useful in the treatment of prion disease. However, further clarification of the distribution of ...

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Bile Acids Reduce Prion Conversion, Reduce Neuronal Loss, and Prolong Male Survival in Models of Prion Disease

Bile Acids Reduce Prion Conversion, Reduce Neuronal Loss, and Prolong Male Survival in Models of Prion Disease

... no disease- modifying therapies available, despite decades of ...neurodegenerative disease models, and we now demonstrate that this effect occurs in prion disease, with an added mechanistic ...

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Familial Prion Disease Cases Without Mutation in PRNPGene

Familial Prion Disease Cases Without Mutation in PRNPGene

... Inherited prion disease, which exhibits autosomal dominant inheritance and which accounts for approximately 15% of human TSEs, is caused by germline mutations in the prion protein ...

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Interaction of prion protein with divalent metals: possible role in neuroprotection and neurodegeneration in prion disease model

Interaction of prion protein with divalent metals: possible role in neuroprotection and neurodegeneration in prion disease model

... of prion disease in human, the genetic component to the disease is most likely the main factor in development of prion ...human prion disease, sources vary from human grafts with ...

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Co-occurrence of chronic traumatic encephalopathy and prion disease

Co-occurrence of chronic traumatic encephalopathy and prion disease

... neurodegenerative disease associated with repetitive traumatic brain injury ...of prion disease in CTE has not been ...National Prion Disease Pathology Surveillance Center of Case ...

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Biochemical Fingerprints of Prion Infection: Accumulations of Aberrant Full-Length and N-Terminally Truncated PrP Species Are Common Features in Mouse Prion Disease

Biochemical Fingerprints of Prion Infection: Accumulations of Aberrant Full-Length and N-Terminally Truncated PrP Species Are Common Features in Mouse Prion Disease

... show symptoms at about 130 to160 days postinfection, and all mice then died within 3 weeks. Brain tissues were obtained from sham-infected mice (n ⫽ 4), mice infected 30 (n ⫽ 4) or 70 days (n ⫽ 4) earlier and not ...

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Prion disease tempo determined by host dependent substrate reduction

Prion disease tempo determined by host dependent substrate reduction

... 10 (versus the abundance of diglycosylated, monoglycosylated, and unglycosylated protein found in disease-free animals; Figure 3A). In the next experiment, we used gel analysis to examine the upper velocity ...

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Immunodeficiency viruses and prion disease

Immunodeficiency viruses and prion disease

... In addition, evidence accumulated that chimpanzees are not suitable as a HIV vaccine model. They could be infected but never progressed to disease. Nevertheless, molecular vi- rology found the reason for this ...

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Test for Detection of Disease-Associated Prion Aggregate in the Blood of Infected but Asymptomatic Animals

Test for Detection of Disease-Associated Prion Aggregate in the Blood of Infected but Asymptomatic Animals

... detect prion aggregates in blood of infected ...additional prion amplification step (Am) and developed a third-generation assay, termed ...detecting disease-associated prion aggregates in ...

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Transmission studies of chronic wasting disease to transgenic mice overexpressing human prion protein using the RT-QuIC assay

Transmission studies of chronic wasting disease to transgenic mice overexpressing human prion protein using the RT-QuIC assay

... wasting disease (CWD) is a fatal prion disease which infects deer, elk and ...human prion protein, have suggested existence of a strong species barrier between cervid CWD and ...human ...

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Journal of Applied Pharmaceutical Science

Journal of Applied Pharmaceutical Science

... cause disease in cattle, sheep, mice, pigs, and mink after intracerebral inoculation (Dawson et ...of prion disease in cattle as they are slaughtered for human ...

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Progress and problems in the biology, diagnostics, and therapeutics of prion diseases

Progress and problems in the biology, diagnostics, and therapeutics of prion diseases

... humans, prion diseases result from infectious modes of transmission (vari- ant Creutzfeldt-Jakob disease [vCJD], iatrogenic CJD, Kuru); inherited modes of transmission in which there is nonconservative germ ...

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Biological network inferences for a protection mechanism against familial Creutzfeldt-Jakob disease with E200K pathogenic mutation

Biological network inferences for a protection mechanism against familial Creutzfeldt-Jakob disease with E200K pathogenic mutation

... Through this study, we confirmed that individuals can have a CJD-free life, even if they harbour the pathogenic E200K mutation, and we provide possible protective me- chanisms to explain this observation. Our research ...

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PubMedCentral-PMC4774245.pdf

PubMedCentral-PMC4774245.pdf

... Japanese prion surveillance work was supported by a grant-in- aid from the Research Committee of Prion Disease and Slow Virus Infection, the Ministry of Health, Labour and Welfare of Japan, and from ...

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