TWiV 950: Prion diseases with Richard Knight

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From the European Society for Clinical Virology 2022 Conference in Manchester UK, Vincent speaks with Richard Knight about prion diseases and the outbreak of bovine spongiform encephalitis that led to cases of variant Creutzfeldt-Jakob disease in humans.

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YouTube timestamps by Les
01:36 guest definition prion disease limited to misfolded prion protein
other neurogenerative diseases seem to involve other misfolded proteins
02:46 diseases with misfolded prion protein
04:01 the 4 ways humans get the disease
Prof Vincent Lecture on topic:
incubation period measured in years
08:07 What makes patients seek a doctor
09:15 diagnosing prion vs other neurological diseases
13:43 what treatments or help is available
17:12 What is the incidence?
18:09 Surveillance
20:40 BSE
37:22 Chronic Wasting disease spreads easily but does it spread to people
39:59 British beef spreading prion disease and how that was stopped
42:25 how to Support this show
Website timestamps by Jolene

lesfaby
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I think this conversation highlights the importance of allowing organisms to eat their species-appropriate diet. Ruminants should eat grass/hay/other cellulose and not processed repurposed animal leftovers or grain for that matter. When ruminants eat what they are meant to eat, their waste will replace the topsoil (that is disappearing at an alarming rate). All farm animals should eat what they are meant to as well as be able to room around in nature interacting with it and eating what they need from it. This is the basis of regenerative farming.

cassandragreer
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Wow, what a great interviewee is Richard Knight: accurate, approachable and fun. This was a great episode. Thanks, TWIV!

claudia
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Geez, I wanted to say Holy Cow after watching this, but my teenage grandchildren warned against it, due to the video subject matter, so I will say Holy Moly. I love these videos immensely, I learn more from Vincent and his team than I’ve learned in 30 years of working in the medical field, and a special shout-out to Daniel Griffin, and of course our lovely Amy! You gentlemen (and ladies) are truly our heroes for bringing us all of this timely information. If nothing else, it lends itself to the age-old advice to enjoy life, it can be cut short in so many, many ways! Vincent’s work is a great cause to contribute to, I hope that everyone will consider a generous donation! We need more science in our world!!

danaweaver
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I'm in Scotland, UK and was given many blood transfusions in 1997, the Scottish blood Bank will not allow me to give blood still and have no plans to change this. There are still putting protections in.

WeePatchesOfLove
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Anyone who talks about the prions is a legend as this field has so many less number of researchers and less volunteers, these guys are really impressive!

THEDIFFERENTONE
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Very interesting interview! I gather the rather distressing disease name “fatal familial insomnia” has been changed? I did not hear it mentioned in the discussion.

sleepydrJ
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Wonderfully different. I became interested in prion diseases after reading an article about them in the NY Times. How awful. Thanks for the update.

susanfleming
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I was working as a junior dr in Cambridge ( UK) in ‘87 on the neurology ward. We had a patient ( who worked at a nearby academic animal research institute) who was ataxic and myoclonic. Investigations back then were limited, and everything came back negative. I was chatting to the professor in charge of the case, and he said ‘ we’ll probably never get a diagnosis, even after post mortem. But i for one have stopped eating lamb’. I asked why. He said ‘scrapie’. So off i went to the library. I too stopped eating lamb.

lulabellegnostic
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This was great introduction to prion diseases. short and to the point.

MI-wcnk
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As a US deer hunter, I've been interested/ worried about "mad deer disease". I suspect a practice that may contribute to its spread: some individual hunters, clubs, commercial hunting operations place "mineral blocks" out as nutrition supplements, to grow larger antlers. Mineral block ingredient lists often include "bone meal", and I am concerned that infectious prions could survive processing and be spread. Mineral blocks are also used in US cattle industry.

larryreily
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I was really impressed and was not expecting this epitope to be that cool and useful! Excellent teaching!

hanscollin
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Thank you for doing this very interesting and informative interview together, Vincent and Richard.

sabine
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Very fascinating discussion. One thing that really interests me is how cjd mimics so many other diseases

lizichell
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28:00 Native Americans used brains as an emulsifying agent in tanning animal skins. Brain tanned leather is something exquisite to the touch. I wonder if prion diseases were found amongst this population, prior to Europeans.

jannamwatson
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Cool. I've been interested in this topic since the outbreaks were big news twenty-five or thirty that's ago.... Thanks!

briancase
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Hi Vincent
Very nice episode
I think protein folding is a very interesting tool to explain such neural disease as explained by your guest
Also i think Dr. Susan lindqest
Explained how chaperone protein Hsp90 playes an important role in the cascade of developing the disease.
Dr Shreiner

shreineraly
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(Previous comment ctd.) If deer can pick up CWD prions from the landscape, what's to prevent cattle, which we eat, from picking up the same prions and developing the same sort of disease, which then becomes a wildfire in the human population? It seems to me only a matter of time before this becomes quite a disaster.

jimmydire
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Fantastic, learned a lot: and I'll never be able to watch 'Bonanza' again without thinking of CWD.

holmbussler
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@V. Great topic and guest. Although we may be eating less meat it is rather concerning that animal organ tissue like brain could end up being used as an emulsifying agent...consumers wouldn't know or question it. We also rely heavily on best practices being used at slaughter houses. One would hope that the future might hold more promise regarding arresting the production of abnormal folding proteins once the disease has been diagnosed thus halting/delaying its progression.

cgmp