References 參考資料： [1] Brown, P., & Bradley, R. (1998). 1755 and all that: a historical primer of transmissible spongiform encephalopathy. BMJ, 317(7174), 1688–1692. https://doi. org/10.1136/bmj.317.7174.1688 [2] Gajdusek, D. C., & Zigas, V. (1957). Degenerative Disease of the Central Nervous System in New Guinea. New England Journal of Medicine, 257(20), 974–978. https://doi.org/nejm195711142572005 [3] Hadlow, W. J. (2008). Kuru likened to scrapie: the story remembered. Philosophical Transactions of the Royal Society of London B: Biological Sciences, 363(1510), 3644. https://doi.org/10.1098/rstb.2008.4013 [4] Hadlow, W. J. (1959). Scrapie and Kuru. Lancet, 274(7097), 289–290. [5] Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019). Kuru, the First Human Prion Disease. Viruses, 11(3), 232. https://doi.org/10.3390/v11030232 [6] Liberski, P. P., Sikorska, B., Lindenbaum, S., Goldfarb, L. G., McLean, C., Hainfellner, J. A., & Brown, P. (2012). Kuru: Genes, Cannibals and Neuropathology. Journal of Neuropathology and Experimental Neurology, 71(2), 92–103. https://doi.org/10.1097/NEN.0b013e3182444efd [7] Quinn, L., Whitfield, J., Alpers, M. P., Campbell, T., Hummerich, H., Pomat, W., Siba, P., Koki, G., Moltke, I., Collinge, J., Hellenthal, G., & Mead, S. (2024). Population structure and migration in the Eastern Highlands of Papua New Guinea, a region impacted by the kuru epidemic. American Journal of Human Genetics, 111(4), 668–679. https://doi.org/10.1016/ j.ajhg.2024.02.011 [8] Alpers M. P. (2008). The epidemiology of kuru: monitoring the epidemic from its peak to its end. Philosophical Transactions of the Royal Society of London B: Biological Sciences, 363(1510), 3707–3713. https://doi.org/10.1098/rstb.2008.0071 [9] Prusiner, S. B. (1998). Prions. Proceedings of the National Academy of Sciences of the United States of America, 95(23), 13363–13383. https://doi.org/10.1073/ pnas.95.23.13363 [10] National Library of Medicine. (2024, June 28). PRNP gene: prion protein (Kanno blood group). MedlinePlus. https://medlineplus.gov/genetics/gene/prnp/ [11] Ramanan, V. K. (2025, February 14). Transmissible Spongiform Encephalopathies. National Institute of Neurological Disorders and Stroke. https://www.ninds. nih.gov/health-information/disorders/transmissiblespongiform-encephalopathies [12] Bernardi, L., & Bruni, A. C. (2019). Mutations in Prion Protein Gene: Pathogenic Mechanisms in C-Terminal vs. N-Terminal Domain, a Review. International Journal of Molecular Sciences, 20(14), 3606. https://doi. org/10.3390/ijms20143606 11 里昂蛋白基因上的突變能引發異常蛋白形成 [12]。 病原性蛋白顆粒的積聚能破壞腦部組織，產生出現 於羊搔癢症、庫魯病和克雅二氏症的海綿狀空洞。與細 菌或病毒等大多數病原體不同，病原性蛋白顆粒並沒有 DNA，所以或許它們感染宿主的背後並不受任何「指令」 指使，而僅為折疊過程中的錯誤，導致這場悲劇。儘管如 此，它們為多個物種帶來無法治癒的致命疾病。 結論 病原性蛋白顆粒的故事見證了科學好奇心、堅持不懈 和合作的力量。最初一個具爭議的假設挑戰了生物學的 基礎法則，儘管看似違反常理，但最終重塑了多個領域 的認知。隨著科學家繼續面對生物學及醫學上的謎團， 這個故事提醒我們：真相並非總是顯而易見，但透過嚴 謹研究、相互合作和敢於挑戰常理的勇氣，即使是最非 比尋常的想法，也能照亮自然中最黑暗的秘密。
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