Prionlar
Özet
Prionların filtre edilebilmeleri ve enfeksiyöz ajan olmaları sebebi ile başlarda virüs oldukları düşünülmüştür. Ancak virion yapılarının veya nükleik asitlerinin (DNA veya RNA) olmaması, formaldehit-yüksek ısı (80°C)-iyonize/ultraviyole ışınlar gibi çeşitli kimyasal ve fiziksel uygulamalara dirençli olmaları ile virüslerden ayrılmaktadırlar (1, 2). Prionlar, merkezi sinir sistemini etkileyen süngerimsi ensefalopatilere neden olan yavaş ilerleyen nörodejeneratif hastalıklara yol açarlar. Bu hastalıklar insanlarda ve hayvanlarda farklı klinik şekillerde görülebilir. İnsanlarda Creutzfeldt-Jacob Hastalığı (CJH), Kuru, Ölümcül Ailesel Uykusuzluk (Fatal Familial Insomnia, FFI) ve Gerstmann-Sträussler-Scheinker Sendromu olarak karşımıza çıkabilmektedir (3). Hayvan hastalıkları arasında ise sığır süngerimsi ensefalopatisi (bovine spongiform encephalopathy, deli dana hastalığı), kaşıntı hastalığı (scrapie, koyun-keçi), kedi süngerimsi ensefalopatileri (feline spongiform encephalopathy), vizonların bulaşıcı ensefalopatisi (transmissible mink encephalopathy) ve kronik zayıflama hastalığı (chronic wasting disease, katır-geyik) yer almaktadır(4).
Referanslar
Fishbein L. Transmissible spongiform encephalopathies, hypotheses and food safety: an overview. Science of the Total Environment. 1998;217(1-2):71-82.
Godon KH, Honstead J. Transmissible spongiform encephalopathies in food animals: human food safety and animal feed safety concerns for veterinarians. Veterinary Clinics of North America: Food Animal Practice. 1998;14(1):49-70.
Collins S, McLean C, Masters C. Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies. Journal of Clinical Neuroscience. 2001;8(5):387-97.
Hüseyinoğlu N. Bulașıcı süngerimsi ensefalopatiler: Halk sağlığı açısından güncel bir bakıș. Kafkas Tıp Bilimleri Dergisi. 2011;1(1):34-40.
Chaudhuri TK, Paul S. Protein‐misfolding diseases and chaperone‐based therapeutic approaches. The Federation of European Biochemical Societies journal. 2006;273(7):1331-49.
Marijanovic Z, Caputo A, Campana V, Zurzolo C. Identification of an intracellular site of prion conversion. Journal of the Public Library of Science pathogens. 2009;5(5):e1000426.
Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216(4542):136-44.
Thackray AM, Knight R, Haswell SJ, Bujdoso R, Brown DR. Metal imbalance and compromised antioxidant function are early changes in prion disease. Biochemical Journal. 2002;362(2):253-8.
Wells G, Hawkins S, Green R, Austin A, Dexter I, Spencer Y, et al. Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Veterinary Record. 1998;142(5):103-6.
Roucou X, LeBlanc AC. Cellular prion protein neuroprotective function: implications in prion diseases. Journal of Molecular Medicine. 2005;83:3-11.
Cohen FE, Prusiner SB. Pathologic conformations of prion proteins. Annual Review of Biochemistry. 1998;67(1):793-819.
Prusiner SB. Neurodegenerative diseases and prions. New England Journal of Medicine. 2001;344(20):1516-26.
Chakraborty C, Nandi S, Jana S. Prion disease: a deadly disease for protein misfolding. Current Pharmaceutical Biotechnology. 2005;6(2):167-77.
Brandel J-P, Culeux A, Grznarova K, Levavasseur E, Lamy P, Privat N, et al. Amplification techniques and diagnosis of prion diseases. Revue Neurologique. 2019;175(7-8):458-63.
Şevik M. Prion Hastalıkları Terapötik Yaklaşımları. Istanbul Medical Journal. 2014;15(2).
Haltia M. Human prion diseases. Annals of medicine. 2000;32(7):493-500.
Jansen C, Van Swieten J, Capellari S, Strammiello R, Parchi P, Rozemuller A. Inherited Creutzfeldt–Jakob disease in a Dutch patient with a novel five octapeptide repeat insertion and unusual cerebellar morphology. Journal of Neurology, Neurosurgery & Psychiatry. 2009;80(12):1386-9.
Ladogana A, Puopolo M, Croes E, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt–Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64(9):1586-91.
Furuya K, Kawahara N, Yamakawa Y, Kishida H, Hachiya NS, Nishijima M, et al. Intracerebroventricular delivery of dominant negative prion protein in a mouse model of iatrogenic Creutzfeldt-Jakob disease after dura graft transplantation. Neuroscience letters. 2006;402(3):222-6.
Collinge J. Variant creutzfeldt-jakob disease. The Lancet. 1999;354(9175):317-23.
Baldelli L, Provini F. Fatal familial insomnia and agrypnia excitata: autonomic dysfunctions and pathophysiological implications. Autonomic Neuroscience. 2019;218:68-86.
Yılmaz H. Prion Hastalıkları-Bulaşabilen Süngerimsi Ensefalopatiler. Ankem Dergisi, İstanbul. 2002.
Madec J, Belli P, Calavas D, Baron T. Efficiency of Western blotting for the specific immunodetection of proteinase K-resistant prion protein in BSE diagnosis in France. The Veterinary Record. 2000;146(3):74-6.
Sakudo A, Ikuta K. Fundamentals of prion diseases and their involvement in the loss of function of cellular prion protein. Protein and peptide letters. 2009;16(3):217-29.
Saá P, Castilla J, Soto C. Presymptomatic detection of prions in blood. Science. 2006;313(5783):92-4.
