Just when you thought it was safe….

…to have a hamburger. ProMED, those wonderful people who brought you Ebola case by case, describe a new way to rot your brain. Read on.

New sporadic prion disease
Date: Fri 13 Aug 2010
Source: Science Daily [edited]


BSE Transmission

New sporadic prion protein disease:
variably protease-sensitive prionopathy shares genotype characteristics with Creutzfeldt-Jakob

A new sporadic prion protein disease has been discovered. Variably protease-sensitive prionopathy (VPSPr), as it has been named, is the 2nd type of complete sporadic disease to be identified since Creutzfeldt-Jakob disease (CJD) was reported in the 1920s. The landmark finding from the National Prion Disease Pathology Surveillance Center at Case Western Reserve University is published in the August [2010] issue of Annals of Neurology [see abstract below].

Normally, the human prion protein gene comes in 3 types due to its
capability to encode prion proteins that contain only the amino acid
methionine, commonly identified as M, both methionine and valine, commonly identified as V, or only for the amino acid valine at position 129. Therefore, when it comes to the prion protein gene unaffected people can be identified as 129MM, 129MV or 129VV. Sporadic CJD (sCJD), which is the most common human prion disease, can affect patients who have any one of the 3 types of the prion protein gene.

In 2008, Pierluigi Gambetti and Wen-Quan Zou, with collaborators, reported the discovery of this novel disease, which affected patients who exhibit only one of the 3 types of the prion protein gene. In this follow-up study, they discovered that all 3 genetic groups can be affected also by this novel disease which now joins sCJD in displaying this feature. However, VPSPr is associated with an abnormal prion protein that exhibits characteristics very different from those of sCJD, as well as other prion diseases, suggesting that it may be caused by a different mechanism, perhaps more akin to other neurodegenerative diseases, such as Alzheimer’s disease. This finding may exemplify, for the 1st time, the possibility that the prion protein affects the brain with different mechanisms.

While examining cases received at the National Prion Disease Pathology Surveillance Center where he is the director, Dr Gambetti observed that a subset of cases had clinical and pathological features quite different from those of all known types of human prion diseases. Further, after being tested for prion proteins via the Western blot [technique] — the gold standard of prion disease diagnosis — the cases were negative. Dr Gambetti then collaborated with Dr Zou, associate director at the center, to solve the riddle of a disease that exhibited some features of a prion disease in histopathological examination but was negative using the standard Western blot test.

Dr Zou’s lab performed a full characterization of the disease and discovered that the VPSPr-associated abnormal prion protein formed a ladder-like electrophoretic profile on Western blotting. “When I obtained the 1st Western blot result of these cases with a different antibody against prions, I was surprised that these cases consistently exhibited this particular profile; one that I had never seen in my more than 10 years of work on human prion diseases,” Dr Zou, assistant professor of pathology at Case Western Reserve School of Medicine, recalls. This ladder-like profile is quite distinctive and very different from the profile of common prion diseases. “Discovery of this unique type of prion provides solid evidence that this novel disease may possess a pathogenesis that is different from that of the major prion diseases currently known,” Dr Zou adds.

Despite extensive research, a relatively large group of neurodegenerative diseases associated with dementia remain undefined. Before being discovered and characterized, VPSPr was one of the undefined dementing diseases. The discovery of VPSPr is chipping away at that group. In the 2 years since its discovery, more than 30 cases have been reported.

“If, as the current evidence indicates, the VPSPr mechanism of affecting the brain is different from that of other sporadic prion diseases, such as sCJD, the discovery of VPSPr would also provide the 1st example that the prion protein may spontaneously damage the brain with different mechanisms,” concludes Dr Gambetti, professor of pathology at Case Western Reserve School of Medicine. “This might apply to other dementing illnesses as well, and has implications for the strategies that need to be followed to attain a cure.”

Drs Gambetti and Zou, along with their extensive research team, plan to further characterize the abnormal prion protein associated with VPSPr as well as other important features of the protein, such as the disease’s propensity for transmission upon inoculation and its replication in test tubes. These features in VPSPr will be compared with those of sCJD to obtain a complete picture of how the abnormal prion protein attacks the brain in these 2 diseases.

- — communicated by: ProMED-mail rapporteur Mary Marshall

[The following is the reference for the paper discussed above followed by the authors' abstract: Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, et al. Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein. Ann Neurol. 2010 Aug;68(2):162-72. ().

Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio.

Abstract

Objective: The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV). Methods: A total of 15 affected subjects with 129MM, 129MV, and 129VV underwent comparative evaluation at the National Prion Disease Pathology Surveillance Center for clinical, histopathologic, immunohistochemical, genotypical, and PrP characteristics. Results: Disease duration (between 22 and 45 months) was significantly different in the 129VV and 129MV subjects. Most other phenotypic features along with the PrP electrophoretic profile were similar but distinguishable in the 3 129 genotypes. A major difference laid in the sensitivity to protease digestion of the disease-associated PrP, which was high in 129VV but much lower, or altogether lacking, in 129MV and 129MM. This difference prompted the substitution of the original designation with "variably protease- sensitive prionopathy" (VPSPr). None of the subjects had mutations in the PrP gene coding region. Interpretation: Because all 3 129 genotypes are involved, and are associated with distinguishable phenotypes, VPSPr becomes the 2nd sporadic prion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920. However, the characteristics of the abnormal prion protein suggest that VPSPr is different from typical prion diseases, and perhaps more akin to subtypes of Gerstmann-Straussler-Scheinker disease.

VPSPr is not linked to eating infected meat. However, like CJD, the new condition happens sporadically. It was 1st identified because of the fast-advancing form of dementia seen in those affected. They were also unable to speak or move. But tests for CJD proved negative. Further molecular examination as described above has shown VPSPr was a prion disease, but one which looked very different to those already known. - Mod.CP]

Enough said.  Just more to worry about…B-)

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7 Responses to “Just when you thought it was safe….”

  1. Just when you thought it was safe…. « ViroBlogy city university Says:

    [...] the original post: Just when you thought it was safe…. « ViroBlogy By admin | category: CASE WESTERN RESERVE University | tags: annals, disease, [...]

  2. Diagnostic Cytology of Koss and its histopathologic bases | Neoplasms Blog Says:

    [...] Just when we suspicion it was safe…. « ViroBlogy [...]

  3. Terry S. Singeltary Sr. Says:

    Tuesday, August 03, 2010

    Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

    http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

    Monday, August 9, 2010

    Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

    http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

    let’s take a closer look at this new prionpathy or prionopathy, and then let’s look at the g-h-BSEalabama mad cow.

    This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ……wait, it get’s better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it’s not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$

    ALABAMA MAD COW g-h-BSEalabama

    In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in “the approximately 10-year-old cow” carrying the E221K mutation.

    http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156

    http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF

    Saturday, August 14, 2010

    BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

    (see mad cow feed in COMMERCE IN ALABAMA…TSS)

    http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

    g-h-BSE-alabama E211K mad cows USA how many would that be annually ???

    if our ciphering is correct (?), that would be about 35 g-h-BSE-alabama E211K mad cows going into the food chain a year.

    an incidence of less than 1 in 2000.

    let’s see, that’s 500 such per million.

    or 50,000 cows per 100 million (US herd size).

    even at less than 1 in a million, with 35 million slaughtered, that’s 35 infected cows going into the food chain each year.

    hmmm, friendly fire there from ???

    Wednesday, July 28, 2010

    re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

    http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html

    Wednesday, August 18, 2010

    Incidence of CJD Deaths Reported by CJD-SS in Canada as of July 31, 2010

    http://creutzfeldt-jakob-disease.blogspot.com/2010/08/incidence-of-cjd-deaths-reported-by-cjd.html

    Monday, August 9, 2010

    National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

    (please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

    http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

    Thursday, August 12, 2010

    USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

    http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html

    14th ICID International Scientific Exchange Brochure –

    Final Abstract Number: ISE.114

    Session: International Scientific Exchange

    Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

    update October 2009

    T. Singeltary

    Bacliff, TX, USA

    Background:

    An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie’s, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

    Methods:

    12 years independent research of available data

    Results:

    I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

    Conclusion:

    I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena’s. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

    http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

    Wednesday, March 31, 2010

    Atypical BSE in Cattle

    http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html

    10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

    Date: March 21, 2007 at 2:27 pm PST

    RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES — CLASS II

    ___________________________________

    PRODUCT

    Bulk cattle feed made with recalled Darling’s 85% Blood Meal, Flash Dried, Recall # V-024-2007

    CODE

    Cattle feed delivered between 01/12/2007 and 01/26/2007

    RECALLING FIRM/MANUFACTURER

    Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

    Firm initiated recall is ongoing.

    REASON

    Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

    VOLUME OF PRODUCT IN COMMERCE

    42,090 lbs.

    DISTRIBUTION

    WI

    ___________________________________

    PRODUCT

    Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI – 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J – PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

    CODE

    The firm does not utilize a code – only shipping documentation with commodity and weights identified.

    RECALLING FIRM/MANUFACTURER

    Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

    REASON

    Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

    VOLUME OF PRODUCT IN COMMERCE

    9,997,976 lbs.

    DISTRIBUTION

    ID and NV

    END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

    http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

    Tuesday, March 2, 2010

    Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA

    http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html

    Monday, March 1, 2010

    ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010

    http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html

    Terry S. Singeltary Sr. (Submitted question): Monday, April 5, 2010

    Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010

    http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html

    TSS

  4. Nursing Care Plan | NCP Creutzfeldt-Jakob Disease | Carcinoma Blog Says:

    [...] Just when we suspicion it was safe…. [...]

  5. Terry S. Singeltary Sr. Says:

    Saturday, December 11, 2010

    Species-barrier-independent prion replication in apparently resistant species

    http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/species-barrier-independent-prion.html

  6. Terry S. Singeltary Sr. Says:

    Thursday, November 18, 2010

    UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS

    http://bse-atypical.blogspot.com/2010/11/united-states-of-america-vs-galen-j.html

  7. Terry S. Singeltary Sr. Says:

    Tuesday, December 14, 2010

    Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J, UPDATE DECEMBER 2010

    http://creutzfeldt-jakob-disease.blogspot.com/2010/12/infection-control-of-cjd-vcjd-and-other.html

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