12 posts · 5,426 views
For clinicians, researchers, and those affected by prion diseases (e.g. Creutzfeldt-Jakob disease)
Brian Appleby
12 posts
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- March 17, 2010
- 09:40 PM
- 377 views
Epidemiology of Human Prion Diseases in the U.S.
by Brian Appleby in CJD Blogger
In January 2010, members of the Centers for Disease Control and Prevention published an article in PLoS One regarding epidemiological data of human prion diseases in the United States. To those who have attended prion meetings in past couple of years, this is not new data as it has been presented before. The study examines the incidence of classical CJD and variant CJD (vCJD) within the U.S. between 1979-2008 and examines some basic demographic features of this population. The data ........ Read more »
Holman, R., Belay, E., Christensen, K., Maddox, R., Minino, A., Folkema, A., Haberling, D., Hammett, T., Kochanek, K., Sejvar, J.... (2010) Human Prion Diseases in the United States. PLoS ONE, 5(1). DOI: 10.1371/journal.pone.0008521
- March 8, 2010
- 10:08 AM
- 325 views
Brief Review of the P-CAPT Filter
by Brian Appleby in CJD Blogger
Following my last post regarding prion blood filtration, I was asked to cover the P-CAPT filter. Because leukoreduction only reduced prion infectivity by 72%, there is a need to develop other ways of eliminating prion infectivity in blood products. The P-CAPT Prion Capture Filter originated from a collaboration with Prometic, the American Red Cross, and several researchers. The majority of the initial research was performed by Luisa Gregori and colleagues in Bob Rohwer's lab, located at the Ve........ Read more »
Gregori, L., Lambert, B., Gurgel, P., Gheorghiu, L., Edwardson, P., Lathrop, J., MacAuley, C., Carbonell, R., Burton, S., Hammond, D.... (2006) Reduction of transmissible spongiform encephalopathy infectivity from human red blood cells with prion protein affinity ligands. Transfusion, 46(7), 1152-1161. DOI: 10.1111/j.1537-2995.2006.00865.x
Gregori L, Gurgel PV, Lathrop JT, Edwardson P, Lambert BC, Carbonell RG, Burton SJ, Hammond DJ, & Rohwer RG. (2006) Reduction in infectivity of endogenous transmissible spongiform encephalopathies present in blood by adsorption to selective affinity resins. Lancet, 368(9554), 2226-30. PMID: 17189034
Murphy CV, Eakins E, Fagan J, Croxon H, & Murphy WG. (2009) In vitro assessment of red-cell concentrates in SAG-M filtered through the MacoPharma P-CAPT prion-reduction filter. Transfusion medicine (Oxford, England), 19(3), 109-16. PMID: 19566667
Wiltshire, M., Thomas, S., Scott, J., Hicks, V., Haines, M., Cookson, P., Garwood, M., & Cardigan, R. (2009) Prion reduction of red blood cells: impact on component quality. Transfusion. DOI: 10.1111/j.1537-2995.2009.02500.x
- February 20, 2010
- 02:37 PM
- 381 views
Feasibility study of a plasma assay for variant CJD-Part 2
by Brian Appleby in CJD Blogger
This post will look at the study conducted by Guntz and colleagues. The authors examined the practical application of a plasma assay for the detection of variant Creutzfeldt-Jakob disease (vCJD) applied to all blood donations. The study intends to study three Blood Transfusion Establishments in France, where 60,000 blood samples will be analyzed over a period of 9-12 months. The current study only reports on the Strasbourg, Alsace site. The Amorfix EP-vCJD blood s........ Read more »
Guntz P, Walter C, Schosseler P, Morel P, Coste J, & Cazenave JP. (2010) Feasibility study of a screening assay that identifies the abnormal prion protein PrP(TSE) in plasma: initial results with 20,000 samples. Transfusion. PMID: 20088835
- January 28, 2010
- 11:31 AM
- 443 views
Presence of prion proteins in non-neuronal tissue in a case of variant CJD
by Brian Appleby in CJD Blogger
A recent article in the journal PLOS One describes a novel finding of abnormal prion proteins (PrPres) in additional non-neuronal tissues of a variant CJD (vCJD) patient. The study comes from the National Prion Disease Pathology Surveillance Center, located at Case Western University in Cleveland, Ohio. Prior to this study, there was evidence that PrPres was present in some non-neuronal tissues as shown below. The present study examined other tissues for the possible presence o........ Read more »
Notari, S., Moleres, F., Hunter, S., Belay, E., Schonberger, L., Cali, I., Parchi, P., Shieh, W., Brown, P., Zaki, S.... (2010) Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States. PLoS ONE, 5(1). DOI: 10.1371/journal.pone.0008765
- January 2, 2010
- 03:30 PM
- 424 views
Prion Propagation: Survival of the Fittest
by Brian Appleby in CJD Blogger
Once again, a post on the evolutionary relevance of prions… As many of you have likely heard, a recent paper in Science describing the evolutionary properties of prions has been published. It’s importance is twofold: 1) the basic understanding that natural selection may extend beyond the genetic code, and 2) important considerations in the development of treatments for prion diseases. The manuscript describes several experiments in which cloned prion strains developed heterogene........ Read more »
Li, J., Browning, S., Mahal, S., Oelschlegel, A., & Weissmann, C. (2009) Darwinian Evolution of Prions in Cell Culture. Science. DOI: 10.1126/science.1183218
- December 19, 2009
- 01:55 PM
- 491 views
Probable vCJD in an individual who was heterozygous at codon 129 of PRNP
by Brian Appleby in CJD Blogger
This week’s Lancet features an article entitled, “Variant CJD in an individual heterozygous for PRNP codon 129” by Kaski and colleagues. The authors report the first case of probable variant Creutzfeldt-Jakob disease (vCJD) in an individual who is heterozygous at codon 129 of the prion protein gene (PRNP). To date, all symptomatic cases have occurred in individuals who are homozygous for methionine at codon 129. As we know from other studies (Brown P, 1994), the incubatio........ Read more »
Kaski, D., Mead, S., Hyare, H., Cooper, S., Jampana, R., Overell, J., Knight, R., Collinge, J., & Rudge, P. (2010) Variant CJD in an individual heterozygous for PRNP codon 129. The Lancet, 374(9707), 2128-2128. DOI: 10.1016/S0140-6736(09)61568-3
- November 24, 2009
- 09:51 AM
- 490 views
The prion protein’s role in neurotransmission
by Brian Appleby in CJD Blogger
As mentioned in a prior post, the exact mechanism of neurotoxicity in prion diseases in unknown. Two possibilities, which are not mutually exclusive, include a loss-of-function of the native prion protein and an acquired neurotoxic effect of the pathologic prion protein. PRNP knockout mice have previously exhibited memory impairment, disruption in circadian rhythms and sleep, behavioral, and neurotransmission changes. Also, excitatory glutamatergic, GABAa receptor-mediated fast........ Read more »
Rangel, A., Madroñal, N., Massó, A., Gavín, R., Llorens, F., Sumoy, L., Torres, J., Delgado-García, J., & Río, J. (2009) Regulation of GABAA and Glutamate Receptor Expression, Synaptic Facilitation and Long-Term Potentiation in the Hippocampus of Prion Mutant Mice. PLoS ONE, 4(10). DOI: 10.1371/journal.pone.0007592
- November 23, 2009
- 11:01 AM
- 538 views
Natural selection among us: The Kuru epidemic
by Brian Appleby in CJD Blogger
Kuru is an acquired prion disease, transmitted through ritualistic cannibalism, that reached epidemic proportions in the Fore tribe of Papua New Guinea. In a previous post, I presented an article by John Collinge’s group on the selection process of heterozygosity at codon 129 of the prion protein gene (PRNP). The research group has gone a step further by recently describing a new polymorphism of the PRNP gene, G129V. The authors performed PRNP genotyping of 3,000 individuals from ........ Read more »
Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Campbell T, Al-Dujaily H, Hummerich H, Beck J, Mein CA.... (2009) A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure. The New England journal of medicine, 361(21), 2056-2065. PMID: 19923577
- November 14, 2009
- 12:25 PM
- 449 views
Prions activate neuronal cholesterogenic gene expression
by Brian Appleby in CJD Blogger
Herman Schatzl, who recently received an endowed chair at the University of Wyoming, and colleagues published an article in The Journal of Biological Chemistry early this year on the effects of prion proteins on the expression of cholesterol-associated genes. The experiment was an in vitro study using cultured neurons that were infected with the 22L prion strain. They examined gene expression of genes involved in the cholesterol pathway in neurons and supportive cells. The authors........ Read more »
Bach C, Gilch S, Rost R, Greenwood AD, Horsch M, Hajj GN, Brodesser S, Facius A, Schädler S, Sandhoff K.... (2009) Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells. The Journal of biological chemistry, 284(45), 31260-9. PMID: 19748890
- November 3, 2009
- 09:54 AM
- 538 views
Components of green tea interfere with the physiologic role of the native prion protein and its conversion into the pathologic prion protein
by Brian Appleby in CJD Blogger
As I continue to catch up on my articles, this post is about an article by Rambold and colleagues that was published in the Journal of Neurochemistry. The researchers’ study was designed to identify compounds that would interfere with the propagation of the pathologic prion protein. The two compounds investigated in the present article were the main components of green tea, epigallocatechin gallate (EGCG) and gallocatechin gallate (GCG). This was an in vitro study that us........ Read more »
Rambold, A., Miesbauer, M., Olschewski, D., Seidel, R., Riemer, C., Smale, L., Brumm, L., Levy, M., Gazit, E., Oesterhelt, D.... (2008) Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP . Journal of Neurochemistry, 107(1), 218-229. DOI: 10.1111/j.1471-4159.2008.05611.x
- November 2, 2009
- 05:05 PM
- 575 views
A layman’s experience of CJD research
by Brian Appleby in CJD Blogger
I happened across a nice little editorial that was published last year in the British Medical Journal that I am saddened to have not seen sooner. It is a piece written by a father of a 25-year-old who died of variant Creutzfeldt-Jakob disease (vCJD). In this short piece, he describes his experience of being involved in several advisory committees on the treatment of human prion diseases as a layperson. I would like to highlight a few of his comments. First, he was surprised to........ Read more »
Firkins, L. (2008) The problem in setting research priorities: a layman's experience. BMJ, 337(jul04 1). DOI: 10.1136/bmj.a212
- October 30, 2009
- 12:08 PM
- 395 views
Anti-prion activity of amphotericin analogues
by Brian Appleby in CJD Blogger
One of several compounds known to inhibit and/or clear pathological prion proteins is amphotericin B. Often called “ampho-terrible” by clinicians because of its toxic side effects, its toxicity has inhibited it from being extensively studied as an anti-prion disease treatment. Its conventional use is as an anti-fungal agent. Soler and colleagues have hence sought to study amphotericin analogues to investigate anti-prion activity in the setting of less toxicity. ........ Read more »
SOLER, L. (2008) Effects of new amphotericin analogues on the scrapie isoform of the prion protein. Biochimica et Biophysica Acta (BBA) - General Subjects, 1780(10), 1162-1167. DOI: 10.1016/j.bbagen.2008.07.005
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