Creutzfeldt-Jakob Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Creutzfeldt-Jakob Disease, including details on mad cow disease, symptoms, causes, variants.

Underestimation of the expression of cellular prion protein on human red blood cells.

Panigaj M, Brouckova A, Glierova H, Dvorakova E, Simak J, Vostal JG, Holada K

From the Institute of Immunology and Microbiology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic; and the Division of Hematology, Center for Biologics Evaluation and Research, FDA, Bethesda, Maryland.

Published 6 May 2011 in Transfusion, 51(5): 1012-21.
Full-text of this article is available online (may require subscription).

Articles on Creutzfeldt-Jakob Disease published 3 May 2011:

Knockdown of prion protein (PrP) by RNA interference weakens the protective activity of wild-type PrP against copper ion and antagonizes the cytotoxicity of fCJD-associated PrP mutants in cultured cells.   Int J Mol Med.

Development of the pathogenesis of transmissible spongiform encephalopathies (TSEs) requires the presence of both the normal host prion protein (PrPC) and the abnormal pathological proteinase-K resistant isoform (PrPSc). Reduction of PrPC levels has been shown to extend survival time after prion infection. In this report, based on analysis of the known sequences of human PrP, we constructed two small interfering RNA (siRNA) duplexes targeting the segments of amino acids (aa) 108-114 (Ri2) and ... [Abstract] [Full-text]

A multistage pathway for human prion protein aggregation in vitro: from multimeric seeds to β-oligomers and non-fibrillar structures.   J Am Chem Soc.

Aberrant protein aggregation causes numerous neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mechanisms remain poorly understood. Here we report AFM results on the formation pathways of β-oligomers and nonfibrillar aggregates from wild type full-length recombinant human prion protein (WT) and an insertion mutant (10OR) with five additional octapeptide repeats linked to familial CJD. Upon partial denaturing, seeds consisting of 3-4 monomers quickly appeared. ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 29 April 2011:

Genetics of Prion Disease.   Top Curr Chem.

Prion diseases or transmissible spongiform encephalopathies (TSEs) are neurodegenerative disorders of humans and animals for which there are no effective treatments or cure. They include Creutzfeldt-Jakob disease (CJD) in humans and sheep scrapie, bovine spongiform encephalopathy (BSE) and chronic wasting disease (CWD) in cervids. The prion protein (PrP) is central to the disease process. An abnormal form of PrP is generally considered to be the sole or principal component of the infectious ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 21 April 2011:

Experience With Preventive Genetic Testing of Corneal Donors in Slovakia.   Cornea.

PURPOSE:: The purpose of this study was (1) to detect asymptomatic carriers of the prion protein gene mutation E200K, which is associated with Creutzfeldt-Jakob disease (CJD), in corneal donors and in the general population of Slovakia and (2) to assess the genetic testing of corneal donors as an effective preventive measure against iatrogenic infection in a country with an unusually high incidence of genetic CJD. METHODS:: The prion protein gene (PRNP) was analyzed in 1133 corneal donors and ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 18 April 2011:

Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease.   Acta Radiol, 52(3): 336-9.

Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three ... [Abstract] [Full-text]

Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease.   Acta Radiol, 52(3): 336-9.

Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three ... [Abstract] [Full-text]

Biological Effects and Use of PrPSc- and PrP-Specific Antibodies Generated by Immunization with Purified Full-Length Native Mouse Prions.   J Virol, 85(9): 4538-46.

The prion agent is the infectious particle causing spongiform encephalopathies in animals and humans and is thought to consist of an altered conformation (PrP(Sc)) of the normal and ubiquitous prion protein PrP(C). The interaction of the prion agent with the immune system, particularly the humoral immune response, has remained unresolved. Here we investigated the immunogenicity of full-length native and infectious prions, as well as the specific biological effects of the resulting monoclonal ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 15 April 2011:

Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant creutzfeldt-jakob disease: implication of small oligomeric species.   Brain Pathol, 21(3): 298-307.

The vacuolation, neuronal loss and gliosis that characterize human prion disease pathology are accompanied by the accumulation of an aggregated, insoluble and protease-resistant form (termed PrP(Sc) ) of the host-encoded normal cellular prion protein (PrP(C) ). In variant Creutzfeldt-Jakob disease the frontal cortex and cerebellum exhibit intense vacuolation and the accumulation of PrP(Sc) in the form of amyloid plaques and plaque-like structures. In contrast the posterior thalamus is ... [Abstract] [Full-text]

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