Research

RESEARCH STUDIES

• Some exciting news on the research work that we have been following with Eric Minikel and Sonia Vallabh:
  Prion disease: potential treatment discovered by researchers and Researchers find new potential treatment for prion diseases

• • Studies Show Pomegranate Supplement Slows Neurodegenerative Diseases (news article link regarding Publication)
  • Delay of gCJD aggravation in sick TgMHu2ME199K mice by combining NPC transplantation and Nano-PSO administration  (pdf of Publication)
    Information provided by Professor Ruth Gabizon from Israel and studies into Pomegranate Seed Oil (PSO) marketed as Granagard.

LAY SUMMARY OF THE PUBLICATION:

Granagard is a nano formulation of PSO (pomegranate seed oil). PSO comprises 80-90% of Punicic Acid (omega 5) which is the strongest natural lipid antioxidant. In vivo, Punicic acid metabolizes into a specific form of conjugated linoleic acid (CLA), which is known to be a calpain inhibitor, suggested as a treatment target for several neurodegenerative conditions. While the active components, both PSO and CLA, do not enter the brain following administration of PSO and actually also CLA, following administration of the Granagard formulation, CLA is found in the brain of rodents, concomitantly with its neuroprotective effect that cannot be found when natural PSO was administrated. Most of our prion related experiments were done in a genetic model of E200K CJD. These mice are born heathy, then start to show neurological disabilities at 5-6 month of age and from there deteriorate until a terminal state at about 12 months of age. They mimic the situation of healthy carriers since they present spontaneous disease caused by a mutant PrP. Continuous Granagard administration from birth or 3 months of age delays the presentation of terminal disease in these mice by almost 6 months. The mechanism of activity of Granagard is most probably related to the maintenance of normal mitochondrial activity even under the stress caused by abnormal protein aggregates, thereby allowing neuronal survival under disease. This is applicable to a number of neurodegenerative disease diseases as well as normal aging. Several human studies are in process (Alzheimer’s disease (AD) ,Parkinson’s disease (PD). Preliminary results in a double-blind study shows that administration of Granagard as compared to placebo improves memory and cognition in MS patients under diverse treatments. As for CJD, we are mostly interested in delay/prevention in asymptomatic carriers of pathological PrP mutations. It is indeed very difficult to establish a proper clinical study to establish if carriers taking Granagard will get sick later or never as compared to those taking placebo. Nobody wants to be in the placebo group for years and most people don’t want to get tested for the mutation. Therefore, we are looking at this in a different manner. Since Granagard is a safe food supplement beneficial to the general public, we follow a large group of genetic families in Israel in which all/most the siblings in affected families are taking it. Then we look, with the help of the medical community in Israel, at the new CJD patients. We believe 30-50% of CJD affected families are taking Granagard regularly. 

We have learned so far that

1: No symptomatic CJD patient has taken Granagard prior to diagnosis.

2: The general number of genetic CJD patients, which was around 20 per year for several years until 2017, has gone down to less than 10 in 2018/2019 and may be as low in 2020.

Obviously, this is all observational and more time is needed to establish if we had affected disease onset, but it looks very encouraging.

• First all-human mouse model of inherited prion disease by Public Library of Science from medicalexpress.com

• From National Institute of Allergies and Infectious Diseases– Eyes of CJD Patients Show Evidence of Prions

• From Neurobiology of Disease– Mitochondrial dysfunction in preclinical genetic prion disease: A target for preventive treatment?

• “The path to an ASO drug for prion disease”. Video Presentation by Eric Minikel and Sonia Vallabh (Broad Institute, Boston, USA) . Originally shown at the 2018 11th Annual National CJD Conference, Melbourne, Australia.

• From La Trobe University- ‘Living’ brain slices reveal how nerve cells die

• Prion protein found in skin of Creutzfeldt-Jakob disease patients: NIH/Case Western Reserve University

• Scientists develop new method to study Creutzfeldt-Jakob disease in the lab

• Research article regarding Jewish New Year fruit from Neurobiology of Disease Volume 108, Issue null, Pages 140-147:
  Continues administration of Nano-PSO significantly increased survival of genetic CJD mice, Orli Binyamin, Guy Keller, Kati Frid, Liraz Larush, Shlomo Magdassi, Ruth Gabizon

• Article from The Times of Israel –Jewish New Year fruit may hold seeds of hope for brain disease sufferers

• Tedx Talk- Dr. Valerie Sim, how her curiosity brought her to discover a new way to bring science to a deeper level.

• Prion Test For Rare, Fatal Brain Disease Might Help Diagnose Alzheimer’s, Parkinson’s

• Prion Test For Rare, Fatal Brain Disease Helps Families Cope

• A new article on Doxycline which we understand was found to be ineffective in prolonging life with CJD patients after several trails. Deana Simpson and I as co-chairs of the CJD International Support Alliance (CJDISA) will ask Professor Inga Zerr, who is a member of the Friends and Advisors Group of the CJDISA for an update. The full article can be found here:  Doxycycline: bringing hope for early sporadic Creutzfeldt-Jakob disease patients

• Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129

• Alzheon Appoints Nobel Laureate Stanley B. Prusiner, MD, as Chair of Scientific Advisory Board

• Blood Tests for Prion Disease

• New detection method could lead to noninvasive diagnosis of Creutzfeldt-Jakob disease

• Gut cells act as gatekeepers against brain diseases

• Impact of Prion Proteins on the Nerves Revealed for the First Time

• Urine test for CJD ‘a possibility’

• Alzheimer’s disease: New drug, detection process could be treatment game changer

• First Glimpse at Infectious Prion Shape

• Newly Discovered Infectious Prion Structure Sheds Light on Mad Cow Disease

• Impact of Prion Proteins on the Nerves Revealed for the First Time

• Blood test could pick up Alzheimer’s disease clues long before symptoms show

• New Anti-Prion Compound Provides New Hope For Treating Brain-Wasting Disease
  

• Paper published by Eric Minikel and Sonia Vallabh: Quantifying prion disease penetrance using large population control cohorts.
  Lay Versions: CureFFI.org: Does This Mean I’ll Definitely Get The Disease?       CureFFI.org: Q&A on genetic prion disease risk

• Dr Brian Appleby from Cleveland USA has provided the first summary which discusses the paper mentioned in the article How Do Prions Kill Brain Cells?

• Professor Will has provided the follow comments for which we are very grateful. We hope this helps reduce any misinterpretation surrounding iatrogenic and sporadic CJD while still maintaining an open mind to all research. New research: sporadic type of Creutzfeldt-Jakob disease (mad cow) results from surgery

• Mad Cow Disease Protein May Play A Role In Depression

• New Anti-Prion Compound Provides New Hope For Treating Brain-Wasting Disease

• The (Mis)diagnosis of Creutzfeldt-Jakob Disease