From Professor Andrew Hill, La Trobe University, Melbourne:
We are proud to share this paper published today from our groups in Melbourne (and collaboration with Inga Zerr in Germany) on identifying potential blood-based biomarkers for CJD. We greatly appreciate the support of the CJDSGN Memorial Awards in memory of Lorraine Seabrook, Adrian Chesterton, Norma Crawley, and Danilo Banzon which helped fund this work. The paper is freely available below.
Prion disease researchers and our CJD Surveillance Unit staff having a Zoom meeting catch-up in honour of CJD international Awareness Day, 12th November, in memory of those lost to CJD in Australia
Publication (pdf)- Delay of gCJD aggravation in sick TgMHu2ME199K mice by combining NPC transplantation and Nano-PSO administration by Kati Frida, Orli Binyamina, Areen Usmana, Ruth Gabizona, Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, Jerusalem, Israel b Medical School, The Hebrew University, Jerusalem, Israel
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.
Some exciting news on the research work that we have been following with Eric Minikel and Sonia Vallabh and Eric Minikel.
From Oxford University Press USA Researchers find new potential treatment for prion diseases
and from healtheuropa.eu Prion disease: potential treatment discovered by researchers
We now have permission to provide this video produced by Eric Minikel and Sonia Vallabh about Antisense Oligonucleotide (ASO) Strategy to reduce PrP that was originally shown at our 2018 Annual National CJD Conference in Melbourne, Australia.
“When illness enters a person’s life, it does not ring the bell, it does not knock on the door, it does not ask for permission. It just comes in. It is silent and discreet, but then suddenly it explodes, devastates, destroys and pulverises everything it encounters on its way.”
Maria Gabriella lost her dad to CJD disease.
Her story and experience of the disease first became a graduation thesis that Maria Gabriella presented a few years ago at an AIEnP meeting dedicated to family members and which today has become a testimony book of dad’s illness experienced and seen through the eyes of a daughter, and the consequences that have affected and marked her family.
Maria Gabriella is a strong woman, with an enormous inner strength. Writing her story has has helped her to overcome the pain that affected her.
Creutzfeldt-Jakob disease. One case in a million
Author: Maria Gabriella Schirinzi
Price: €17 plus shipping