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The Disease You Will Never Survive

2023-02-10

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Check out DeepMind's AlphaFold - and marvel at the complex 3D structure of proteins - at https://alphafold.ebi.ac.uk/

A simple mis-folding in a certain brain protein causes a disease for which we have no cure.

LEARN MORE

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To learn more about this topic, start your googling with these keywords:

- Prion: an abnormal pathogenic agent that is transmissible and able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain.

- Spongiform encephalopathies: a group of rare degenerative brain disorders caused by prions and characterized by tiny holes that give the brain a “spongy” appearance.

- Amyloid: an abnormal aggregate of protein.

- Creutzfeldt-Jakob disease: also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is an invariably fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis.

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CREDITS

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Lizah van der Aart | Script Writing, Narration, Illustration, Video Editing and Animation

Kate Yoshida | Script Editor and Video Director

Nathaniel Schroeder | Music

A huge thanks to Dr. Emmanuelle Vire for sharing her expertise on prion biology and epigenetics.

MinuteEarth is produced by Neptune Studios LLC

https://neptunestudios.info

OUR STAFF

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Lizah van der Aart • Sarah Berman • Cameron Duke

Arcadi Garcia i Rius • David Goldenberg • Melissa Hayes

Alex Reich • Henry Reich • Peter Reich

Ever Salazar • Leonardo Souza • Kate Yoshida

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REFERENCES

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Dabin LC, Guntoro F, Campbell T, Bélicard T, Smith AR, Smith RG, Raybould R, Schott JM, Lunnon K, Sarkies P, Collinge J, Mead S, Viré E (2020). Altered DNA methylation profiles in blood from patients with sporadic Creutzfeldt-Jakob disease. Acta Neuropathology 140(6):863-879. https://doi.org/10.1007/s00401-020-02224-9

Miller G. (2009) Neurodegeneration. Acting like a prion isn't always bad. Science 326 (5958):1338. https://doi.org/10.1126/science.326.5958.1338

Quartararo, AJ. (2020) Synthesis of Proteins by Automated Flow Chemistry. Science 368 (6494): 980– 987, https://doi.org/10.1126/science.abb2491

Sabate R. (2014) When amyloids become prions. Prion 8 (3):233-9. https://doi.org/10.4161/19336896.2014.968464

Scheckel, C., Aguzzi, A. (2018) Prions, prionoids and protein misfolding disorders. Nature Reviews Genetics 19, 405–418. https://doi.org/10.1038/s41576-018-0011-4

Vastag, B. (2009) The beneficial side of prions. Nature https://doi.org/10.1038/news.2009.23


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