Navigation Links
New sporadic prion protein disease identified by Case Western Reserve
Date:8/13/2010

A new sporadic prion protein disease has been discovered. Variably protease-sensitive prionopathy (VPSPr), as it has been named, is the second 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 issue of Annals of Neurology.

Normally, the human prion protein gene comes in three 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 three types of the prion protein gene. In 2008, Pierluigi Gambetti, MD, and Wen-Quan Zou, MD, PhD, with collaborators, reported the discovery of this novel disease, which affected patients who exhibit only one of the three types of the prion protein gene. In this follow-up study, they discovered that all three 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 first 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 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 blot. "When I obtained the first 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 two 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 first 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 two diseases.


'/>"/>

Contact: Jessica Studeny
jessica.studeny@case.edu
216-368-4692
Case Western Reserve University
Source:Eurekalert

Related medicine news :

1. Veterans Affairs Researchers Induce a New Transmissible Prion Disease
2. Prion Disease in Mice May Help Advance Alzheimers Research
3. New and Delicious, Almond Butter Filled, Cookie Bites With 35.7% Protein to Help Manage Weight and Build Muscle
4. Research highlights role of protein pair in obesity regulation
5. Urine protein test might help diagnose kidney damage from lupus, UT Southwestern researchers find
6. Smithfield, United Food and Commercial Workers Union, and Food Networks Paula Deen to Deliver 150,000 Servings of Protein to San Francisco Food Bank
7. Protein Sciences Corporation Announces Profitable and Cash Flow Positive Results for 2009 and Management Realignment
8. Protein Appears Key to Intestinal Balance
9. SIBLING proteins may predict oral cancer
10. Damaged protein identified as early diagnostic biomarker for Alzheimers disease in healthy adults
11. Cells of aggressive leukemia hijack normal protein to grow
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/22/2017)... (PRWEB) , ... February 22, 2017 , ... ... its certification exam and eligibility requirements effective with the April 2017 testing period. ... 1995. , “The Care Manager Certified (CMC) exam is periodically re-calibrated to ...
(Date:2/22/2017)... ... February 22, 2017 , ... In a ... skipping doses or not filling a prescription because they could not afford to ... problems getting medications were 30-60%*. At the same time, hospitals, pharmacies, ...
(Date:2/22/2017)... Colorado (PRWEB) , ... February ... ... the availability of their newest advanced absorption cannabidiol (CBD) serum, “NANOCALM 300” ... delivery system®, which provides instant absorption from the mouth into the bloodstream. ...
(Date:2/22/2017)... WV (PRWEB) , ... February 22, 2017 , ... ... awarded a contract to Quality Insights to help small practices in Delaware, New ... Payment Program, established by the Medicare Access and CHIP Reauthorization Act of 2015 ...
(Date:2/22/2017)... ... February 22, 2017 , ... Michael ... new media platform connecting healthcare technology professionals and other key stakeholders with an ... quarterly publication starting on March 1, announced Michael J. Hennessy, Jr., president of ...
Breaking Medicine News(10 mins):
(Date:2/22/2017)... -- Andre, DiMino, CEO of ADM Tronics Unlimited, Inc. (OTCQB: ... and products, commented on ADMT,s third quarter fiscal year ... the Company,s quarterly report on Form 10Q available at ... stated "During the quarter ended December 31, 2016 we ... portion of our engineering efforts into the development of ...
(Date:2/22/2017)... , Feb. 22, 2017 Summary ... infarction partnering deals and agreements entered into by ... full report: http://www.reportlinker.com/p03605670-summary/view-report.html Description The ... understanding and access to partnering deals and agreements ... - Trends in partnering deals ...
(Date:2/22/2017)... 22, 2017 Summary Provides ... partnering deals and agreements entered into by the ... report: http://www.reportlinker.com/p03605673-summary/view-report.html Description The Global ... understanding and access to partnering deals and agreements ... - Trends in partnering deals ...
Breaking Medicine Technology: