An overview of human prion diseases

Virology Journal - Tập 8 Số 1 - 2011
Muhammad Imran1, Saqib Mahmood2
1Department of Physiology and Cell Biology, Centre for Research in Endocrinology and Reproductive Sciences (CRERS), University of Health Sciences (UHS), Lahore, 54600, Pakistan
2Department of Human Genetics and Molecular Biology, University of Health Sciences (UHS), Lahore, 54600, Pakistan

Tóm tắt

Từ khóa


Tài liệu tham khảo

Linden R, Martins VR, Prado MAM, Cammarota M, Izquierdo I, Brentani RR: Physiology of the prion protein. Physiol Rev 2008, 88: 673-728. 10.1152/physrev.00007.2007

Pan KM, Baldwin M, Nguyen J, Gasset M, Serban A, Groth D, Mehlhorn I, Huang Z, Fletterick RJ, Cohen FE, Prusiner SB: Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci USA 1993, 90: 10962-10966. 10.1073/pnas.90.23.10962

Prusiner SB: Prions. Proc Natl Acad Sci USA 1998, 95: 13363-13383. 10.1073/pnas.95.23.13363

Smirnovas V, Baron GS, Offerdahl DK, Raymond GJ, Caughey B, Surewicz WK: Structural organization of brain-derived mammalian prions examined by hydrogen-deuterium exchange. Nat Struct Mol Biol 2011, 18: 504-506. 10.1038/nsmb.2035

Zahn R, Liu A, Luhrs T, Riek R, von Schroetter C, Lopez Garcia F, Billeter M, Calzolai L, Wider G, Wuthrich K: NMR solution structure of the human prion protein. Proc Natl Acad Sci USA 2000, 97: 145-150. 10.1073/pnas.97.1.145

Collinge J, Clarke AR: A general model of prion strains and their pathogenicity. Science 2007, 318: 930-936. 10.1126/science.1138718

Aguzzi A: Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis. J Neurochem 2006, 97: 1726-1739. 10.1111/j.1471-4159.2006.03909.x

Imran M, Mahmood S: An overview of animal prion diseases. Virol J 2011, 8: 493. 10.1186/1743-422X-8-493

Marsh RF, Hadlow WJ: Transmissible mink encephalopathy. Rev Sci Tech 1992, 11: 539-550.

Sigurdson CJ, Miller MW: Other animal prion diseases. Brit Med Bull 2003, 66: 199-212. 10.1093/bmb/66.1.199

Williams ES, Young S: Chronic wasting disease of captive mule deer: a spongiform encephalopathy. J Wildl Dis 1980, 16: 89-98.

Wells GAH, Scott AC, Johnson CT, Gunning RF, Hancock RD, Jeffrey M, Dawson M, Bradley R: A novel progressive spongiform encephalopathy in cattle. Vet Rec 1987, 121: 419-420. 10.1136/vr.121.18.419

Kirkwood JK, Cunningham AA, Wells GA, Wilesmith JW, Barnett JE: Spongiform encephalopathy in a herd of greater kudu (Tragelaphus strepsiceros): epidemiological observations. Vet Rec 1993, 133: 360-364. 10.1136/vr.133.15.360

Kirkwood JK, Cunningham AA: Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Vet Rec 1994, 135: 296-303. 10.1136/vr.135.13.296

Wyatt JM, Pearson GR, Gruffydd-Jones TJ: Feline spongiform encephalopathy. Feline Pract 1993, 21: 7-9.

Bons N, Mestre-Frances N, Belli P, Cathala F, Gajdusek DC, Brown P: Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents. Proc Natl Acad Sci USA 1999, 96: 4046-4051. 10.1073/pnas.96.7.4046

Gajdusek DC, Zigas V: Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of "kuru" in the native population. New Engl J Med 1957, 257: 974-978. 10.1056/NEJM195711142572005

Creutzfeldt HG: U" ber eine eigenartige herdfo"rmige Erkrankung des Zentralnervensystems. Z Gesamte Neurol Psychiatr 1920, 57: 1-19. 10.1007/BF02866081

Jakob A: U" ber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswertem anatomischem. Befunde. (Spastische Pseudosklerose-Encephalomyelopathie mit disseminierten Degenerationsherden). Z Gesamte Neurol Psychiatr 1921, 64: 147-228. 10.1007/BF02870932

Kirschbaum WR: Zwei eigenartige Erkrankung des Zentralnervensystems nach Art der spatischen Pseudosklerose (Jakob). Z Neurol Pyschiatry 1924, 92: 175-220. 10.1007/BF02877841

Richardson EP, Masters CL: The nosology of Creutzfeldt-Jakob disease and conditions related to the accumulation of PrPCJD in the nervous system. Brain Pathol 1995, 5: 33-41. 10.1111/j.1750-3639.1995.tb00575.x

Duffy P, Wolf J, Collins G, DeVoe AG, Streeten B, Cowen D: Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974, 290: 692-693.

Lugaresi E, Medori R, Montagna P, Baruzzi A, Cortelli P, Lugaresi A, Tinuper P, Zucconi M, Gambetti P: Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. N Engl J Med 1986, 315: 997-1003. 10.1056/NEJM198610163151605

Will RG, Ironside JW, Zeidler M, Cousens SM, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG: A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996, 347: 921-925. 10.1016/S0140-6736(96)91412-9

Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB: Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med 1999, 340: 1630-1638. 10.1056/NEJM199905273402104

Gambetti P, Dong Z, Yuan J, Xiao X, Zheng M, Alshekhlee A, Castellani R, Cohen M, Barria MA, Gonzalez-Romero D, Belay ED, Schonberger LB, Marder K, Harris C, Burke JR, Montine T, Wisniewski T, Dickson DW, Soto C, Hulette CM, Mastrianni JA, Kong Q, Zou WQ: A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 2008, 63: 697-708. 10.1002/ana.21420

Mead S, Stumpf MP, Whitfield J, Beck J, Poulter M, Campbell T, Uphill J, Goldstein D, Alpers MP, Fisher E, Collinge J: Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 2003, 300: 640-643. 10.1126/science.1083320

Belay ED: Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999, 53: 283-314. 10.1146/annurev.micro.53.1.283

Brandel JP: Transmissible spongiform encephalopathies. Orphanet Encyclopedia 2004, 1-7. [ http://www.orpha.net/data/patho/GB/uk-TSE.pdf ]

Kawauchi Y, Kamitani T, Yagishita S, Kitamoto T, Kishida H: Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum. Neuropathology 2006, 26: 313-317. 10.1111/j.1440-1789.2006.00683.x

Cornelius JR, Boes CJ, Ghearing G, Leavitt JA, Kumar N: Visual symptoms in the Heidenhain variant of Creutzfeldt-Jakob Disease. J Neuroimaging 2009, 19: 283-287. 10.1111/j.1552-6569.2008.00294.x

Parchi P, Strammiello R, Giese A, Kretzschmar H: Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future. Acta Neuropathol 2011, 121: 91-112. 10.1007/s00401-010-0779-6

Court L, Bert J: Electrophysiologie des encéphalopathies transmissibles. Path Biol 1995, 43: 25-42.

Bratosiewicz-Wasik J, Liberski PP, Golanska E, Jansen GH, Wasik TJ: Regulatory sequences of the PRNP gene influence susceptibility to sporadic Creutzfeldt-Jakob disease. Neurosci Lett 2007, 411: 163-167. 10.1016/j.neulet.2006.08.001

Mead S, Mahal SP, Beck J, Campbell T, Farrall M, Fisher E, Collinge J: Sporadic--but not variant--Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. Am J Hum Genet 2001, 69: 1225-1235. 10.1086/324710

Palmer MS, Dryden AJ, Hughes JT, Collinge J: Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 1991, 352: 340-342. 10.1038/352340a0

Windl O, Dempster M, Estibeiro JP, Lathe R, De Silva R, Esmonde T, Will R, Springbett A, Campbell TA, Sidle KCL, Palmer MS, Collinge J: Genetic basis of Creutzfeldt-Jakob disease in the United Kingom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene. Hum Genet 1996, 98: 259-264. 10.1007/s004390050204

Shibuya S, Higuchi J, Shin R-W, Tateishi J, Kitamoto T: Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease. Lancet 1998, 351: 419.

Shibuya S, Higuchi J, Shin RW, Tateishi J, Kitamoto T: Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1998, 43: 826-828. 10.1002/ana.410430618

Soldevila M, Calafell F, Andrés AM, Yagüe J, Helgason A, Stefánsson K, Bertranpetit J: Prion susceptibility and protective alleles exhibit marked geographic differences. Hum Mutat 2003, 22: 104-104.

Brandner S: Diversity of prion diseases: (no) strains attached? Acta Neuropathol 2011, 121: 1-4. 10.1007/s00401-010-0775-x

Gambetti P, Cali I, Notari S, Kong Q, Zou WQ, Surewicz WK: Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol 2011, 121: 79-90. 10.1007/s00401-010-0761-3

Wadsworth JDF, Collinge J: Molecular pathology of human prion disease. Acta Neuropathol 2011, 121: 69-77. 10.1007/s00401-010-0735-5

Capellari S, Strammiello R, Saverioni D, Kretzschmar H, Parchi P: Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis. Acta Neuropathol 2011, 121: 21-37. 10.1007/s00401-010-0760-4

Montagna P, Gambetti P, Cortelli P, Lugaresi E: Familial and sporadic fatal insomnia. Lancet Neurol 2003, 2: 167-176. 10.1016/S1474-4422(03)00323-5

Baldin E, Capellari C, Provini F, Corrado P, Liguori R, Parchi P, Montagna P, Cortelli P: A case of fatal familial insomnia in Africa. J Neurol 2009, 256: 778-1779.

Gambetti P, Kong Q, Zou W, Parchi P, Chen SG: Sporadic and familial CJD: classification and characterization. Brit Med Bull 2003, 66: 213-239. 10.1093/bmb/66.1.213

Parchi P, Capellari S, Chin S, Schwarz HB, Schecter NP, Butts JD, Hudkins P, Burns DK, Powers JM, Gambetti P: A subtype of sporadic prion disease mimicking fatal familial insomnia. Neurology 1999, 52: 1757-1763.

Priano L, Giaccone G, Mangieri M, Albani G, Limido L, Brioschi A, Pradotto L, Orsi L, Mortara P, Fociani P, Mauro A, Tagliavini F: An atypical case of sporadic fatal insomnia. J Neurol Neurosurg Psychiatry 2009, 80: 924-927. 10.1136/jnnp.2008.154815

Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ: Molecular cloning of a human prion protein cDNA. DNA 1986, 5: 315-324. 10.1089/dna.1986.5.315

Nozaki I, Hamaguchi T, Sanjo N, Noguchi-Shinohara M, Sakai K, Nakamura Y, Sato T, Kitamoto T, Mizusawa H, Moriwaka F, Shiga Y, Kuroiwa Y, Nishizawa M, Kuzuhara S, Inuzuka T, Takeda M, Kuroda S, Abe K, Murai H, Murayama S, Tateishi J, Takumi I, Shirabe S, Harada M, Sadakane A, Yamada M: Prospective 10-year surveillance of human prion diseases in Japan. Brain 2010, 133: 3043-3057. 10.1093/brain/awq216

Corsaro A, Thellung S, Bucciarelli T, Scotti L, Chiovitti K, Villa V, D'Arrigo C, Aceto A, Florio T: High hydrophobic amino acid exposure is responsible of the neurotoxic effects induced by E200K or D202N disease-related mutations of the human prion protein. Int J Biochem Cell Biol 2011, 43: 372-382. 10.1016/j.biocel.2010.11.007

Nitrini R, Rosemberg S, Passos-Bueno MR, Teixeira da Silva LS, Iughetti P, Papadopoulos M, Carrilho PM, Caramelli P, Al- brecht S, Zatz M, LeBlanc A: Familial spongiform encephalopathy with distinct clinico-pathological features associated with a novel prion gene mutation at codon 183. Ann Neurol 1997, 42: 138-146. 10.1002/ana.410420203

Takazawa T, Ikeda K, Ito H, Aoyagi J, Nakamura Y, Miura K, Iwamoto K, Kano O, Kawabe K, Iwasaki Y: A distinct phenotype of leg hyperreflexia in a Japanese family with Gerstmann-Sträussler-Scheinker syndrome (P102L). Intern Med 2010, 49: 339-342. 10.2169/internalmedicine.49.2864

Provini F, Vetrugno R, Pierangeli G, Cortelli P, Rizzo G, Filla A, Strisciuglio C, Gallassi R, Montagna P: Sleep and temperature rhythms in two sisters with P102L Gerstmann-Stra"ussler-Scheinker (GSS) disease. Sleep Med 2009, 10: 374-377. 10.1016/j.sleep.2008.03.004

Bianca M, Bianca S, Vecchio I, Raffaele R, Ingegnosi C, Nicoletti F: Gerstmann-Sträussler-Scheinker disease with P102L-V129 mutation: a case with psychiatric manifestations at onset. Ann Genet 2003, 46: 467-469. 10.1016/S0003-3995(03)00017-0

Jansen C, Parchi P, Capellari S, Vermeij AJ, Corrado P, Baas F, Strammiello R, van Gool WA, van Swieten JC, Rozemuller AJ: Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP. Acta Neuropathol 2010, 119: 189-197. 10.1007/s00401-009-0609-x

Alzualde A, Indakoetxea B, Ferrer I, Moreno F, Barandiaran M, Gorostidi A, Estanga A, Ruiz I, Calero M, van Leeuwen FW, Atares B, Juste R, Rodriguez-Martínez AB, López de Munain A: A novel PRNP Y218N mutation in Gerstmann-Sträussler-Scheinker disease with neurofibrillary degeneration. J Neuropathol Exp Neurol 2010, 69: 789-800. 10.1097/NEN.0b013e3181e85737

Liberski PP, Brown P: Kuru: its ramifications after fifty years. Exp Gerontol 2009, 44: 63-69. 10.1016/j.exger.2008.05.010

Will RG: Acquired prion disease: iatrogenic CJD, variant CJD, kuru. Brit Med Bull 2003, 66: 255-265. 10.1093/bmb/66.1.255

Collinge J, Palmer MS, Dryden AJ: Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 1991, 337: 1441-1442. 10.1016/0140-6736(91)93128-V

Gajdusek DC, Gibbs CJ, Alpers M: Experimental transmission of a kuru-like syndrome to chimpanzee. Nature 1966, 209: 794-796. 10.1038/209794a0

Brandner S, Whitfield J, Boone K, Puwa A, O'Malley C, Linehan JM, Joiner S, Scaravilli F, Calder I, Alpers MP, Wadsworth JDF, Collinge J: Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. Phil Trans R Soc B 2008, 363: 3755-3763. 10.1098/rstb.2008.0091

Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP: Kuru in the 21st century -- an acquired human prion disease with very long incubation periods. Lancet 2006, 367: 2068-2074. 10.1016/S0140-6736(06)68930-7

Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Campbell T, Al-Dujaily H, Hummerich H, Beck J, Mein CA, Verzilli C, Whittaker J, Alpers MP, Collinge J: A novel protective prion protein variant that colocalizes with Kuru exposure. N Engl J Med 2009, 361: 2056-2065. 10.1056/NEJMoa0809716

Wadsworth JDF, Joiner S, Linehan JM, Desbruslais M, Fox K, Cooper S, Cronier S, Asante EA, Mead S, Brandner S, Hill AF, Collinge J: Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Nat Acad Sci USA 2008, 105: 3885-3890. 10.1073/pnas.0800190105

Manuelidis L, Chakrabarty T, Miyazawa K, Nduom NA, Emmerling K: The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents. Proc Natl Acad Sci USA 2009, 106: 13529-13534. 10.1073/pnas.0905825106

Manuelidis L: Transmissible encephalopathy agents: virulence, geography and clockwork. Virulence 2010, 1: 101-104. 10.4161/viru.1.2.10822

Beekes M: Variant Creutzfeldt-Jakob disease (vCJD): epidemiology and prevention from human to human secondary transmission. Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 2010, 53: 597-605. 10.1007/s00103-010-1070-7

Lukic A, Beck J, Joiner S, Fearnley J, Sturman S, Brandner S, Wadsworth JD, Collinge J, Mead S: Heterozygosity at polymorphic codon 219 in variant Creutzfeldt-Jakob disease. Arch Neurol 2010, 67: 1021-1023. 10.1001/archneurol.2010.184

Ironside JW, Bishop MT, Connolly K, Hegazy D, Lowrie S, Le Grice M, Ritchie DL, McCardle LM, Hilton DA: Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 2006, 332: 1164-1165. 10.1136/bmj.332.7551.1164

Bishop MT, Pennington C, Heath CA, Will RG, Knight RS: PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism. BMC Med Genet 2009, 10: 146. 10.1186/1471-2350-10-146

Wadsworth JDF, Asante EA, Desbruslais M, Linehan JM, Joiner S, Gowland I, Welch J, Stone L, Lloyd SE, Hill AF, Brandner S, Collinge J: Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science 2004, 306: 1793-1796. 10.1126/science.1103932

Asante EA, Linehan JM, Gowland I, Joiner S, Fox K, Cooper S, Osiguwa O, Gorry M, Welch J, Houghton R, Desbruslais M, Brandner S, Wadsworth JDF, Collinge J: Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice. Proc Natl Acad Sci USA 2006, 103: 10759-10764. 10.1073/pnas.0604292103

Hizume M, Kobayashi A, Teruya K, Ohashi H, Ironside JW, Mohri S, Kitamoto T: Human prion protein (PrP) 219K is converted to PrPSc but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection. J Biol Chem 2009, 284: 3603-3609.

Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Hewitt P, Collinge J: Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 2006, 368: 2061-2067. 10.1016/S0140-6736(06)69835-8

Wilson K, Ricketts MN: A new human genotype prone to variant Creutzfeldt-Jakob disease. BMJ 2006, 332: 1164-1165. 10.1136/bmj.332.7551.1164

Kaski D, Mead S, Hyare H, Cooper S, Jampana R, Overell J, Knight R, Collinge J, Rudge P: Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 2009, 374: 2128. 10.1016/S0140-6736(09)61568-3

Brown P, Preece M, Brandel JP, Sato T, McShane L, Zerr I, Fletcher A, Will RG, Pocchiari , Cashman NR, d'Aignaux JH, Cervena'kova' L, Fradkin J, Schonberger LB, Collins SJ: Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000, 55: 1075-1081.

Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R, Notari S, Crain B, Schmidt RE, Geschwind M, Dearmond SJ, Cairns NJ, Dickson D, Honig L, Torres JM, Mastrianni J, Capellari S, Giaccone G, Belay ED, Schonberger LB, Cohen M, Perry G, Kong Q, Parchi P, Tagliavini F, Gambetti P: Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol 2010, 68: 162-172. 10.1002/ana.22094

Jansen C, Head MW, van Gool WA, Baas F, Yull H, Ironside JW, Rozemuller AJ: The first case of protease-sensitive prionopathy (PSPr) in The Netherlands: a patient with an unusual GSS-like clinical phenotype. J Neurol Neurosurg Psychiatry 2010, 81: 1052-1055. 10.1136/jnnp.2009.175646

Rodríguez-Martínez AB, Garrido JM, Zarranz JJ, Arteagoitia JM, de Pancorbo MM, Atarés B, Bilbao MJ, Ferrer I, Juste RA: A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: case report. BMC Neurol 2010, 10: 99. 10.1186/1471-2377-10-99

Norrby E: Prions and protein-folding diseases. J Intern Med 2011, 270: 1-14. 10.1111/j.1365-2796.2011.02387.x

Imran M, Mahmood S, Hussain R, Abid NB, Lone KP: Frequency distribution of PRNP polymorphisms in the Pakistani population. Gene 2012, 492: 186-194. 10.1016/j.gene.2011.10.029

Yu SL, Jin L, Sy MS, Mei FH, Kang SL, Sun GH, Tien P, Wang FS, Xiao GF: Polymorphisms of the PRNP gene in Chinese populations and the identification of a novel insertion mutation. Eur J Hum Genet 2004, 12: 867-870. 10.1038/sj.ejhg.5201245

Grasbon-Frodl E, Schmalzbauer R, Weber P, Krebs B, Windl O, Zerr I, Kretzschmar HA: A novel three extra-repeat insertion in the prion protein gene (PRNP) in a patient with Creutzfeldt-Jakob disease. Neurogenetics 2004, 5: 249-250. 10.1007/s10048-004-0196-x

Nishida Y, Sodeyama N, Toru Y, Toru S, Kitamoto T, Mizusawa H: A case of Creutzfeldt-Jakob disease with a novel insertion mutation and codon 219 Lysine/Lysine polymorphism in the prion protein gene. Prions 2005, 251-252.