My Photo
Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Monday, September 19, 2016

Evidence of scrapie transmission to sheep via goat milk

Evidence of scrapie transmission to sheep via goat milk


Timm KonoldEmail author, Leigh Thorne, Hugh A. Simmons, Steve A. C. Hawkins, Marion M. Simmons and Lorenzo González


BMC Veterinary ResearchBMC series – open, inclusive and trusted201612:208


DOI: 10.1186/s12917-016-0807-4


© Crown copyright; licensee BioMed Central Ltd. 2016


Received: 29 April 2016


Accepted: 19 August 2016


Published: 17 September 2016






Previous studies confirmed that classical scrapie can be transmitted via milk in sheep. The current study aimed to investigate whether scrapie can also be transmitted via goat milk using in vivo (new-born lambs fed milk from scrapie-affected goats due to the unavailability of goat kids from guaranteed scrapie-free herds) and in vitro methods (serial protein misfolding cyclic amplification [sPMCA] on milk samples).




In an initial pilot study, new-born lambs of two different prion protein gene (PRNP) genotypes (six VRQ/VRQ and five ARQ/ARQ) were orally challenged with 5 g brain homogenate from two scrapie-affected goats to determine susceptibility of sheep to goat scrapie. All sheep challenged with goat scrapie brain became infected based on the immunohistochemical detection of disease-associated PrP (PrPsc) in lymphoid tissue, with an ARQ/ARQ sheep being the first to succumb. Subsequent feeding of milk to eight pairs of new-born ARQ/ARQ lambs, with each pair receiving milk from a different scrapie-affected goat, resulted in scrapie in the six pairs that received the largest volume of milk (38–87 litres per lamb), whereas two pairs fed 8–9 litres per lamb, and an environmental control group raised on sheep milk from healthy ewes, did not show evidence of infection when culled at up to 1882 days of age. Infection in those 12 milk recipients occurred regardless of the clinical status, PrPsc distribution, caprine arthritis-encephalitis virus infection status and PRNP polymorphisms at codon 142 (II or IM) of the donor goats, but survival time was influenced by PRNP polymorphisms at codon 141. Serial PMCA applied to a total of 32 milk samples (four each from the eight donor goats collected throughout lactation) detected PrPsc in one sample each from two goats.




The scrapie agent was present in the milk from infected goats and was able to transmit to susceptible species even at early preclinical stage of infection, when PrPsc was undetectable in the brain of the donor goats. Serial PMCA as a PrPsc detection method to assess the risk of scrapie transmission via milk in goats proved inefficient compared to the bioassay.


Keywords Transmissible spongiform encephalopathy – Scrapie – Goat – Sheep – Milk – Colostrum – Transmission – Protein misfolding cyclic amplification – Prion protein – Genotype


see full text ;





***Importantly, infectious prions are present in the brain, bodily fluids and excreta of animals showing no clinical signs of TSE [32]–[37]. For example, the blood, saliva and urine from deer subclinically infected with CWD contain infectious prions [32], [38], [39]. Race and coworkers [40] found a long-term subclinical carrier state in mice infected with hamster scrapie. Atypical BSE (BASE) can be transmitted from non-clinical cattle to primates [41] and cattle exposed to large oral doses of BSE can remain free of clinical signs but are found to harbor infectious prions upon bioassay into bovid PrP transgenic mice [42]. Taken together, these findings suggest that carriers without clinical signs exist and may transmit infection to their offspring via milk, transplacental trafficking or blood.




Sun Jan 14, 2007 20:5771.248.156.194


Subject: Prion Protein in Milk Date: January 14, 2007 at 5:41 pm PST PLoS ONE. 2006; 1(1): e71. Published online 2006 December 20. doi: 10.1371/journal.pone.0000071. Copyright Franscini et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Prion Protein in Milk


Nicola Franscini,1 Ahmed El Gedaily,1 Ulrich Matthey,1 Susanne Franitza,1 Man-Sun Sy,2 Alexander Bürkle,3 Martin Groschup,4 Ueli Braun,5 and Ralph Zahn1*


1Alicon AG, Schlieren, Switzerland 2Institute of Pathology, Biomedical Research Building, Case Western University School of Medicine, Cleveland, Ohio, United States of America 3Lehrstuhl Molekulare Toxikologie, University of Konstanz, Konstanz, Germany 4Friedrich-Loeffler-Institut, Bundesforschungsinstitut für Tiergesundheit, Greifswald, Gemany 5Departement für Nutztiere, University of Zurich, Zurich, Switzerland Matthew Baylis, Academic Editor


University of Liverpool, United Kingdom


 * To whom correspondence should be addressed. E-mail: Conceived and designed the experiments: ZR FN EA MU FS RZ NF AE UM SF. Performed the experiments: FN EA MU FS NF AE UM SF. Analyzed the data: ZR FN EA MU FS RZ NF AE UM SF. Contributed reagents/materials/analysis tools: AB MG SM BA GM BU MS UB. Wrote the paper: AB MG ZR FN BA GM BU RZ NF UB.


Received October 19, 2006; Accepted November 6, 2006.




Background Prions are known to cause transmissible spongiform encephalopathies (TSE) after accumulation in the central nervous system. There is increasing evidence that prions are also present in body fluids and that prion infection by blood transmission is possible. The low concentration of the proteinaceous agent in body fluids and its long incubation time complicate epidemiologic analysis and estimation of spreading and thus the risk of human infection. This situation is particularly unsatisfactory for food and pharmaceutical industries, given the lack of sensitive tools for monitoring the infectious agent.


Methodology/Principal Findings We have developed an adsorption matrix, Alicon PrioTrap®, which binds with high affinity and specificity to prion proteins. Thus we were able to identify prion protein (PrPC)–the precursor of prions (PrPSc)–in milk from humans, cows, sheep, and goats. The absolute amount of PrPC differs between the species (from µg/l range in sheep to ng/l range in human milk). PrPC is also found in homogenised and pasteurised off-the-shelf milk, and even ultrahigh temperature treatment only partially diminishes endogenous PrPC concentration.


Conclusions/Significance In view of a recent study showing evidence of prion replication occurring in the mammary gland of scrapie infected sheep suffering from mastitis, the appearance of PrPC in milk implies the possibility that milk of TSE-infected animals serves as source for PrPSc.








Milk contributes 13% to the worldwide protein supply for humans. World milk production ranges around 500 million tons a year. Before fresh milk reaches the consumer, it is usually homogenized to reduce fat particle size in order to increase digestibility of the milk and heated. Surprisingly, pasteurisation (heating for 30 seconds to 72°C) and ultra-high temperature treatment (heating for 1–4 seconds to 135°C) only leads to a partial reduction of the amount of PrPC. This supports the observation that PrPC is highly stable in milk. Thus, the heating procedures used to inactivate DNA-containing pathogens are not sufficient to eliminate endogenous prion proteins.


The presence of PrPC in blood has been documented [14], [15], and is confirmed by our own unpublished observations. To produce one liter of milk, about 400 to 500 liters of blood must pass through the udder of a cow. It is thus possible that the PrPC found in milk derives from blood cells or, alternatively, has been secreted from glandular epithelial cells. Cell types that have been identified in milk from healthy cows are mainly macrophages, and other leucocytes. However, in our assay cells are completely removed by centrifugation. Therefore, the recovered PrPC is not cell associated but most likely binds to other proteins or lipids resulting in stable molecular complexes. The fact that milk contains full-length PrPC, very likely comprising the glycolipid anchor, indicates that prion protein was originally cell-bound and does not represent any of the amino-terminal truncation products of PrPC known to be released from normal cells under physiological conditions [25]. The detection of such a considerable expression of cell membrane bound or derived PrPC in milk constitutes one of the key requirements for the generation of infectious prions in the udder of infected animals.


Over the last 10 years, scientific groups, risk assessment agencies, and public health organizations have debated the TSE risk for milk and milk products [26], [27]. Epidemiological and bioassay data so far available have not provided evidence for milk to harbour prion infectivity and infectious prions have as yet not been detected by bioassays in the milk, colostrum or udder of clinical BSE cases in cow [18]–[21]. However, a recent statement of the European Food Safety Authority affirmed that based on a number of observations from research data, there are indications that infectivity in the milk from small ruminants can not be totally excluded [28]. Furthermore, the exclusion of animals with mastitis, an inflammation of the mammal gland, being able to destabilize the blood-milk barrier, is considered a measure able to reduce but not to eliminate the potential contamination risk [28]. The rational of this conclusion is confirmed by a recent study showing that in sheep naturally affected with both scrapie and lymphocyte or lymphofollicular mastitis, PrPSc accumulation was present in lymphoid follicles adjacent to milk ducts [29]. At least in natural sheep scrapie, prion replication can occur following a lymphotropic virus infection in the inflamed mammary gland. This study has not detected PrPSc or prion infectivity in milk itself. However, since under such inflammatory conditions, the total number of immune cells increases in milk of animals, it might be possible that infectious PrPSc is also passing through and reaches the milk. In this context milk from such animals could possibly be responsible for the spread of scrapie from the ewes to their offspring in affected sheep or goat flocks. Moreover, sheep and goat milk could also constitute a TSE exposure risk for mammals (humans) consuming these products.


The former Scientific Steering committee of the European Commission and the European Food Safety Authority recommend that research should intensify on the safety of milk of small ruminants with regard to TSE risk. Limited new data are expected to be published in the near future and there is still little research initiated in this area [28]. The Alicon PrioTrap® technology opens a new avenue for studying the biochemical characteristics of prion protein in milk and thus may contribute to offer a feasible approach to perform an appropriate study on the milk safety with regard to TSE risk.







 Re: PRION PROTEIN IN MILK Mon Jan 15, 2007 12:19


> Thus we were able to identify prion protein (PrPC)–the precursor of


> prions (PrPSc)–in milk from humans, cows, sheep, and goats.




> Conclusions/Significance


> In view of a recent study showing evidence of prion replication occurring in the mammary gland of scrapie


> infected sheep suffering from mastitis, the appearance of PrPC in milk implies the possibility that milk of


> TSE-infected animals serves as source for PrPSc.


 TO DATE, BSE has not been detectable in milk. NOW, what about BASE and or atypical TSE in bovine? well, that's another question. and with testing in the pipeline that is much more sensistive, yet to be validated, low level TSE infectivity in milk is still very much possible. then, what about accumulation ? well, we all can hope that we die from old age first. PrPSc HAS been detected in mammary glands of sheep affected by scrapie and mastitis. ...tss


studies of interest ;


SEAC 90th meeting held on the 24th Nov $ THE vCJD EPIDEMIC Thu Dec 1, 2005 10:23




© SEAC 2005






The Spongiform Encephalopathy Advisory Committee held its 90th


meeting in Edinburgh on 24th November 2005, and discussed the


following matters:




SEAC was informed about the following issues:


• The SEAC Sheep Subgroup will meet on 24th January 2006


to consider emerging scientific developments on atypical


scrapie and possible implications for the National Scrapie


Plan (NSP) and EU TSE roadmap.


• A recent article1 reporting detection of relatively low levels of


abnormal prion in the mammary glands of five Italian farmed


sheep with coincident clinical signs of natural scrapie and


mastitis. Abnormal prion protein was absent in the


mammary gland of sheep free of mastitis or scrapie. SEAC


agreed that the study provided further evidence that


inflammatory diseases can alter the distribution of abnormal


prion protein in infected animals. However, the particular


form of mastitis was rare in the UK and the sheep breed


studied is prone to particular diseases. SEAC considered


the study to be important. However, it would be premature to


come to firm conclusions about the possible implications of


the findings for UK flocks and further investigations should


be undertaken. It was noted that regulations restrict milk


from animals with clinical mastitis from entering the food




1 Ligios et al. (2005) PrPSc in mammary glands of sheep affected by scrapie


and mastitis. Nature Medicine, 11 published online 04/11/05.




© SEAC 2005






 see full text here ;


 PrPSc in mammary glands of sheep affected by scrapie and mastitis. Ligios C, Sigurdson CJ, Santucciu C, Carcassola G, Manco G, Basagni M, Maestrale C, Cancedda MG, Madau L, Aguzzi A.





 "Occurrence and Stability of the BSE Agent in Foodstuff (primarily in Milk and Milk Products) and in the Environment" For the protection of the consumer a scientifically based risk assessment of animal derived products, particularly milk, meat and products thereof is of prime importance. The knowledge on BSE in this regard is insufficient and also this topic is at present not taken into account in the EU projects on transmissible spongiform encephalopathies. It is also unknown at present, if and to what degree the BSE prions, considered to be very stable, can be degraded or inactivated by microbial processes in the gastrointestinal tract of animals, by fermentation processes during food processing and by the complex microflora of the environment. The project therefore aims at the improvement of food safety as well as the protection of the consumer and the environment. In order to create the scientific base for a quantitative risk assessment, the following tasks will be solved using modern trace analytical, microbiological and molecular biological methods.


1) Sensitive detection methods for PrPSc in bovine body fluids shall be developed with the main emphasis on the detection of small amounts of the agent in milk but also in blood and meat dripping juice.


2) The microbial degradation of PrPSc shall be studied in order to gain basic knowledge on the stability and fate of the BSE agent in the gastrointestinal tract, in the environment (soil) and in foodstuff (milk and meat products).




Scientific basis for a rik assessment for food





Project partners:


Bundesanstalt für Fleischforschung - Institut für Mikrobiologie und Toxikologie TU München - Institut für Mikrobiologie, Forschungszentrum für Milch und Lebensmittel Weihenstephan BFAV - Institut für neue und neuartige Tierseuchenerreger LMU München - Lehrstuhl für Hygiene und Technologie der Milch Publications:


Biochemical evidence for the proteolytic degradation of infectious prion protein PrPSc in hamster brain homogenates by foodborne bacteria Müller-Hellwig S, Groschup MH, Pichner R, Gareis M, Märtlbauer E, Scherer S, Loessner MJ Syst Appl Microbiol 2006 Mar; 29 (2): 165-71


Degradation of scrapie associated prion protein (PrPSc) by the gastrointestinal microbiota of cattle Christina Scherbel, Rohtraud Pichner, Matin H.Groschup, Simone Mueller.Hellwig, Siegfried Scherer, Richard Dietrich, Erwin Maertlbauer, Manfred Gareis Vet Res. 37 (2006) 695-703


Cellular Prion Protein in the Bovine Mammary Gland Is Selectively Expressed in Didier A, Dietrich R, Steffl M, Gareis M, Groschup MH, Muller-Hellwig S, J Histochem Cytochem. 2006 Jul 24; [Epub ahead of print]


Prion protein expression in bovine podocytes and extraglomerular mesangial Amselgruber WM, Steffl M, Didier A, Martlbauer E, Pfaff E, Buttner M. Cell Tissue Res. 2006 Jun;324(3):497-505. Epub 2006 Feb 17


Relevant Branches:


food technology health





Minutes of the open session of the 88th meeting held on 30th June 2005




(SEAC 88/2)


starts at page 6 of 21.......tss





Risk of TSE infection through milk There never has been any evidence of milk as a method of transfer of scrapie and experiments in which a ewe with scrapie was suckled by the offspring of another sheep showed no indication that any risk was present. However, it would be surprising if there was no infection present in milk at all simply because of the number of white cells that are present in the milk. At the moment there are no useful inoculation studies published that look for milk infectivity adequately.




No evidence for BSE transmission through milk


(17.5.99 as a press release by Consumer Safety and Food)


The Scientific Steering Committee (SSC) sees no evidence for transmission of BSE through milk. Hence, there is no reason for restriction on the use of milk, stated the 16 independent scientists in an opinion on possible vertical transmission of BSE (from mother to calf) on Friday. The higher infection rate of calves from BSE-infected cows of between 5 and 15% is rather to be found in other routes of contagion. The definite mechanisms for vertical transmission still need to be clarified, they said. With respect to these mechanisms, the SSC considers that transmission of BSE by artificial insemination is unlikely for semen derived from BSE-affected bulls early in their incubation period and that transmission of BSE via embryos is unlikely provided International Embryo Transfer Society protocols are used.


However, the experts stated that - as a precautionary measure - milk from BSE-affected cows should be taken out, although this milk is not proven to be infectuous. The safety of milk is permanently monitored by the SSC. The Committee also put a preliminary opinion on the recycling of fallen stock, dead animals (including also ruminants, pigs, poultry, fish, wild/zoo/exotic animals, laboratory animals, fish, fur animals and cats) and condemned materials, i.e. animals not fit for human consumption onto internet for comments of interested parties. With respect to the high risks of TSEs and unconventional, not yet identified infectious agents, the present industrial processes of inactivating conventional infectious agents could not be considered safe enough. The scientists recommend that animals and SRMs with TSE or suspected of being so, should be incinerated or burned after rendering and not be recycled for any direct or indirect use, including use in cosmetics, pharmaceutical or medical products and devises. The opinion and the preliminary opinion will shortly be available under the following address :


 However one of the additions to the SSC report was: "Regarding inoculating cattle i/c with milk, the following comment can be made: In practice it might prove to be more appropriate / useful to look (also) at certain fractions of milk and to use transgenic mice rather than cattle once the model is proven. The aim of the experiments should be to improve the confidence that milk and colostrum do not transmit BSE. Colostrum is more important in the context of protecting animal health and eventually eliminating BSE and thus a BSE source for humans."




 Safety of milk with regard to TSE: State of affairs (From Food Safety announcement, 2001) 1. Summary


a. Because of the recent evidence of BSE being present in a number of countries where it had not been detected until recently, Commission services are frequently invited to express their opinion on the security status of ruminant milk as a possible source of TSE infectivity.


b. The experimental evidence so far of bovine milk being safe with regard to BSE risk has been questioned because these experiments were carried out on mice; it was considered1 that these tests may have underestimated any possible risk because of the species barrier from cows to mice. It is noted that milk had the potential to transmit prion diseases like BSE because it contains a significant component of leucocytes.


The Commission services therefore invited the Scientific Steering Committee (SSC) to prepare a state of affairs regarding the available knowledge and evidence on the safety of milk with regard to TSE risk.


On the basis of the discussion of a report prepared by the TSE/BSE ad hoc Group, the Scientific Steering Committee (SSC) considers that the conclusions of the Scientific Veterinary Committee (E.C., 1996), the Multidisciplinary Scientific Committee (E.C., 1997) and itself (E.C., 1999), remain valid and that the evidence available to date does not point at milk or colostrum representing a possible risk. The SSC also supports the recommendation that for precautionary reasons the milk, colostrum or milk products from suspect BSE cases should not be offered for consumption.


No final results of further experiments became recently available and the SSC confirms the recommendations for research made in each of the previously listed scientific opinions.


2. Report from the TSE/BSE ad hoc Group


The TSE/BSE ad hoc Group reviewed the information and evidence available to date. The state-of-affairs report prepared at its meeting of 15 March 2001 on the basis of contributions from various scientists (see section 3) is presented hereafter.


2.1 The Report of the Scientific Veterinary Committee of 1996 (E.C., 1997)


In its opinion of 1996, the Scientific Veterinary Committee, "having examined the considerable epidemiological and infectivity data pertaining to BSE, maternal transmission and milk, concludes that bovine milk from healthy cows and products derived therefrom which contain no animal-derived additives can be safely consumed in any form by any species. There is no evidence that milk transmits BSE and the Committee considers any risk from milk to be negligible".


"In regard to colostrum, [the Committee] regarded any risks to be negligible." It further recommended a "Prohibition on the use of milk (or colostrum) or any product derived from these commodities from any cow clinically suspected to have BSE for any nutritional or other purpose. An exception for the suspect's own calf can be made" (...).


2.2. The scientific opinion of the Multidisciplinary Scientific Committee of 1997 (Will, 1997, E.C., 1997b).


The evidence contained in the full report of the Scientific Veterinary Committee (E.C., 1996) was reviewed in 1997 by the Multidisciplinary Scientific Committee (Will, 1997; E.C., 1997) that concurred with the 1996 conclusions.


A maternal cohort study provided statistical evidence of a maternal risk enhancement (e.g., Donnelly et al, 1997a, b)2, but did not provide evidence on the risk of transmission by milk as the calves in this study received only colostrum from their dams and as other explanations than vertical transmission (for example peri-natal conditions) could not be excluded.


A serious hint that milk does not transmit the disease was the beef suckler study (Wilesmith and Ryan, 1997, 1998; Donnelly, 1998) where no cases were reported although all of them received colostrum and milk.


2.3. The SSC opinion on vertical transmission (1999)


In its opinion of 18-19 March 1999 on The possible vertical transmission of Bovine Spongiform Encephalopathy (BSE), the SSC referred as follows to the work of the UK Spongiform Encephalopathy Advisory Committee (SEAC):


"As regards the risks from bovine milk, the Scientific Steering Committee refers to the continuous review by the UK Spongiform Encephalopathy Advisory Committee (SEAC). SEAC has regularly discussed the safety of bovine milk in regard to BSE, the last time on 9 November 1998. The latest substantive SEAC view, expressed on 16 April 1997, was that the measures currently in place to protect the consumer were considered appropriate. (UK law states that milk derived from BSE affected cattle or cattle suspected to have BSE shall not be sold, supplied or used for human or animal consumption, with the exception that it may be fed to the cow's own calf.) SEAC concluded then (16/4/97) that no evidence had been found to suggest that milk from any species affected by transmissible spongiform encephalopathies was infectious. The Committee is keeping the possible risk infectivity in milk under review and stated most recently on 14 May 1998 that there was no reason to change their previous advice on the safety of milk. This advice may need to be updated as new data and information become available.


However, the Scientific Steering Committee notes that, in the absence of any infectivity studies on semen, embryos, fetal tissue, milk and colostrum by i/c inoculation of the homologous species in bovines, ovines and caprines, and in the absence of all the necessary experimental and epidemiological data as detailed in the report, precise estimates of these risks cannot be made."


2.4. Summary of presently available evidence (March 2001):


a. To date, the infectious agent has not been detected by mice bioassay of milk from humans with kuru or CJD or from cattle with BSE3 or sheep with scrapie.


b. To date, no mother to child transmissions have occurred in any animal species used in experimental studies (including primates).


c. One single case has been reported on in 1992 of a 38-year-old pregnant woman with sporadic CJD whose colostrum was found to be infected when injected i/c into mice (Tamai et al, 1992). However, following further morphological examination of fixed mouse brain by immunohistochemistry for PrPSc , the Japanese authorities concluded tha






Sun Jan 14, 2007 20:5871.248.156.194


Immunochemical PrPC detection


Concentrated milk and brain PrPC was denaturated in SDS sample buffer and heated at 70°C for 10 min and at 95°C for 5 min, respectively. Samples were applied to a 12% SDS polyacrylamide gel for electrophoresis and subsequently transferred to a PVDF membrane. The membrane was blocked with 2% ECL Advance™ blocking agent (Amersham) if probed with 8B4 antibody or 1% bovine serum albumin if probed with other antibodies used in this study. 8B4 antibody was incubated at a concentration of 450 ng/ml, 8H4 at 85 ng/ml, 6H4 at 285 ng/ml, and anti-Tau-1 at 50 ng/ml. Sheep anti-mouse IgG horseradish peroxidase-conjugated secondary antibody (Amersham) was incubated at a 1/20,000 dilution. The immunoreactivity was visualized by chemiluminesecence detection following the manufacture's instruction (ECL Advance Western Blot detection Kit, Amersham).


PNGase treatment of milk and brain PrPC For PNGase treatment prion protein extracted from 10 ml cow milk or 10 µl of 1% (w/v) cow brain homogenate was incubated for 12 h at 37°C in buffer containing 100 mM sodium phosphate, 10 mM Tris-HCl, 1% NP-40, 1% MEGA-8, pH 8, and 1.5 units of N-Glycosidase F (Roche). Under more stringent cleavage conditions, proteins were denatured by heating for 10 minutes at 100°C in the presence of 0.5% SDS before treatment with 4 units of N-Glycosidase F.


Total milk protein detection A milk volume corresponding to 40 µg total protein (1 µl) was heated in SDS loading buffer at 70°C for 10 min. After electrophoresis on a 12% SDS polyacrylamide gel, the proteins were detected by silver staining (SilverSNAP Stain kit II, Pierce).




We thank Theo Bearth, Roland Ermini, Peter May, Günter Kreissel, and Mathias Schmid for their generous support. We are grateful to Torsten Seuberlich from the NeuroCenter of the University of Bern for kindly providing us brain homogenate from cattle.




Competing Interests: The authors have declared that no competing interests exist. Funding: The authors have no support or funding to report.




1.Bolton DC, McKinley MP, Prusiner SB. Identification of a protein that purifies with the scrapie prion. Science. 1982;218:1309–1311. [PubMed] 2.Prusiner S B, et al. Further purification and characterization of scrapie prions. Biochemistry. 1982;21:6942–6950. 3.Oesch B, Westaway D, Walchli M, McKinley MP, Kent SB, et al. A cellular gene encodes scrapie PrP 27–30 protein. Cell. 1985;40:735–746. [PubMed] 4.Basler K, Oesch B, Scott M, Westaway D, Walchli M, et al. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell. 1986;46:417–428. [PubMed] 5.Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216:136–144. [PubMed] 6.Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. Prion protein gene variation among primates. J Mol Biol. 1995;245:362–374. [PubMed] 7.Kretzschmar HA, Prusiner SB, Stowring LE, DeArmond SJ. Scrapie prion proteins are synthesized in neurons. Am J Pathol. 1986;122:1–5. [PubMed] 8.Mobley WC, Neve RL, Prusiner SB, McKinley MP. Nerve growth factor increases mRNA levels for the prion protein and the β-amyloid protein precursor in developing hamster brain. Proc Natl Acad Sci USA. 1988;85:9811–9815. [PubMed] 9.Manson J, West JD, Thomson V, McBride P, Kaufman MH, et al. The prion protein gene: a role in mouse embryogenesis? Development. 1992;115:117–122. [PubMed] 10.Turk E, Teplow DB, Hood LE, Prusiner SB. Purification and properties of the cellular and scrapie hamster prion proteins. Eur J Biochem. 1988;176:21–30. [PubMed] 11.Stahl N, Borchelt DR, Hsiao K, Prusiner SB. Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell. 1987;51:229–240. [PubMed] 12.Haraguchi T, Fisher S, Olofsson S, Endo T, Groth D, et al. Asparagine-linked glycosylation of the scrapie and cellular prion proteins. Arch Biochem Biophys. 1989;274:1–13. [PubMed] 13.Cashman NR, Loertscher R, Nalbantoglu J, Shaw I, Kascsak RJ, et al. Cellular isoform of the scrapie agent protein participates in lymphocyte activation. Cell. 1990;61:185–192. [PubMed] 14.MacGregor I, Hope J, Barnard G, Kirby L, Drummond O, et al. Application of a time-resolved fluoroimmunoassay for the analysis of normal prion protein in human blood and its components. Vox Sang. 1999;77:88–92. [PubMed] 15.Barclay GR, Houston EF, Halliday SI, Farquhar CF, Turner ML. Comparative analysis of normal prion protein expression in human, rodent, and ruminant blood cells by using a panel of prion antibodies. Transfusion. 2002;42:517–526. [PubMed] 16.Ironside JW, Head MW. Variant Creutzfeldt-Jakob disease: risk of transmission by blood and blood products. Haemophilia. 2004;10(Suppl 4):64–69. [PubMed] 17.Castilla J, Saa P, Soto C. Detection of prions in blood. Nat Med. 2005;11:982–985. [PubMed] 18.Hadlow WJ, Kennedy RC, Race RE, Eklund CM. Virologic and neurohistologic findings in dairy goats affected with natural scrapie. Vet Pathol. 1980;17:187–199. [PubMed] 19.Bradley R, Wilesmith JW. Epidemiology and control of bovine spongiform encephalopathy (BSE). Br Med Bull. 1993;49:932–959. [PubMed] 20.Taylor DM, Ferguson CE, Bostock CJ, Dawson M. Absence of disease in mice receiving milk from cows with bovine spongiform encephalopathy. Vet Rec. 1995;136:592. [PubMed] 21.Buschmann A, Groschup MH. Highly bovine spongiform encephalopathy-sensitive transgenic mice confirm the essential restriction of infectivity to the nervous system in clinically diseased cattle. J Infect Dis. 2005;192:934–942. [PubMed] 22.Li R, Liu T, Wong BS, Pan T, Morillas M, et al. Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol. 2000;301:567–573. [PubMed] 23.Korth C, Stierli B, Streit P, Moser M, Schaller O, et al. Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature. 1997;390:74–77. [PubMed] 24.Zanusso G, Liu D, Ferrari S, Hegyi I, Yin X, et al. Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci USA. 1998;95:8812–8816. [PubMed] 25.Laffont-Proust I, Faucheux BA, Hassig R, Sazdovitch V, Simon S, et al. The N-terminal cleavage of cellular prion protein in the human brain. FEBS Lett. 2005;579:6333–6337. [PubMed] 26.EC. Scientific Veterinary Committee, Report on the risk analysis for colostrium, milk, and milk products (Document N° VI/8197/96 Version J, Final, 1997). 27.EC. Multidisciplinary Scientific Committee, Opinion on the possible risks related to the use of colostrium, milk and products (1997). 28.Statement of the European Food Safety Authority Scientific Expert Working Group on BSE/TSE of the Scientific Panel on Biological Hazards on the health risks of the consumption of milk and milk derived products from goats (2004). Last updated 11. Juli 2006. Available: Accessed 2006 Oct 16. 29.Ligios C, Sigurdson CJ, Santucciu C, Carcassola G, Manco GC, et al. PrPSc in mammary glands of sheep affected by scrapie and mastitis. Nat Med. 2005;11:1137–1138. [PubMed]





Monday, September 05, 2016


*** Pathological features of chronic wasting disease in reindeer and demonstration of horizontal transmission Major Findings for Norway



Wednesday, September 7, 2016


*** An assessment of the long-term persistence of prion infectivity in aquatic environments ***



Sunday, August 24, 2008


HAVE ANOTHER GLASS OF CWD PRIONS COURTESY Dane County Wisconsin Mike DiMaggio, solid waste manager



Friday, September 02, 2016


Chronic Wasting Disease Drives Population Decline of White-Tailed Deer



Saturday, September 17, 2016


Texas Parks Wildlife Chronic Wasting Disease CWD Management and Regulations for Hunters 2016 – 2017



Monday, August 29, 2016





Sunday, August 28, 2016




Transmissible Spongiform Encephalopathy TSE Prion and how Politics and Greed by the Industry spread madcow type diseases from species to species and around the globe






Saturday, December 12, 2015


NOTICE: Environmental Impact Statement on Large Livestock Carcasses TSE Prion REPORT December 14, 2015



Friday, August 14, 2015


Carcass Management During a Mass Animal Health Emergency Draft Programmatic Environmental Impact Statement—August 2015




food for thought, specified risk materials srm’s, TSE Prions anyone...


Thursday, August 25, 2016


FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA MAD COW TYPE DISEASE



Tuesday, December 16, 2014


Evidence for zoonotic potential of ovine scrapie prions


Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics




Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.


Subject terms: Biological sciences• Medical research At a glance



*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.


*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.


*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.



Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online


Taylor & Francis


Prion 2016 Animal Prion Disease Workshop Abstracts


WS-01: Prion diseases in animals and zoonotic potential


Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,


Natalia Fernandez-Borges a. and Alba Marin-Moreno a


"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France


Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.


To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.


These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.


Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.




why do we not want to do TSE transmission studies on chimpanzees $


5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.











Saturday, April 23, 2016


*** SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016 ***


Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X



Transmission of scrapie prions to primate after an extended silent incubation period


***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***



Thursday, August 18, 2016





Tuesday, August 9, 2016


*** Concurrence with OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2015-0055]



Saturday, July 23, 2016





Tuesday, July 26, 2016


*** Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016



Saturday, July 16, 2016


*** Importation of Sheep, Goats, and Certain Other Ruminants [Docket No. APHIS-2009-0095]RIN 0579-AD10


WITH great disgust and concern, I report to you that the OIE, USDA, APHIS, are working to further legalize the trading of Transmissible Spongiform Encephalopathy TSE Pion disease around the globe.


THIS is absolutely insane. it’s USDA INC.



*** Specified Risk Materials SRMs BSE TSE Prion Program ***



Thursday, October 22, 2015


*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened those mad cows in Texas ***



*** Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle ***


Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.




The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...





In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells


3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...



”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” 26.






Post a Comment

Subscribe to Post Comments [Atom]

<< Home