Bovine spongiform encephalopathy: epidemiology and diagnosis
Povzetek
Goveja spongioformna encefalopatija (BSE): epidemiologija in diagnoze. Goveja spongioformna encefalopatija (BSE) spada v skupino tako imenovanih "prionskih bolezni". Za to smrtno nevrodegenerativno bolezen so značilne dolge inkubacijske dobe in v klinični fazi prisotnost amiloidnih depozitov v centralnem živčnem sistemu ter nenormalno zavita oblika proteina PrP. BSE se je pojavila leta 1986 v Veliki Britaniji, verjetno s pomočjo krmljenja živine z živalskimi beljakovinami iz okuženih trupov ovac okuženih s praskavcem. BSE je bil izvožen v druge Evropske države preko MBM in žive živine, vendar pa je bila najverjetneje prisotna že preje pri ljudeh z varianto Creutzfeldt-Jakobove bolezni (CJB). Prvi takšen primer je bil opisan v Veliki Britaniji leta 1996. Pojavnosti CJB se redno povečuje v večini primerov med državljani Združenega kraljestva. Zaradi težke klinične diagnoze je pojavnost BSE v državah izven VB bila verjetno podcenjena. Nekatere države (Nemčija, Španija, Italija) so do leta 2001 prezrle primere BSE. Testi, ki jih je EU odobrila temeljijo na odkrivanju nenormalnih PrP (proteinaze K odporen) v možganih govedi po zakolu. Ti testi zagotavljajo razumen previdnostni ukrep za zaščito zdravja ljudi (najboljši varnostni ukrep pa je odstranitev specifičnega rizičnega materiala iz trupov), vendar to ne more veliko pomagati pri izkoreninjenju BSE. Nadzor in izkoreninjenje BSE bosta odvisna od posebnih in občutljivih diagnostičnih testov, ki se lahko uporabljajo na živih živali v začetku okužbe. Več nedavno objavljenih rezultatov daje upanje, da bi lahko v bližnji prihodnosti imeli na voljo takšne zgodnje diagnostične teste.
Prenosi
Literatura
Braun U, Schicker E, Hornlimann B. Diagnostic reliability of clinical signs in cows with suspected bovine spongiform encephalopathy. Vet Rec. 1998;143:101-5.
Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, BOVINE SPONGIFORM ENCEPHALOPATHY 6 McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H,Bostock CJ. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997;389:498-501.
Carp RI, Meeker HC, Rubenstein R, Sigurdarson S, Papini M, Kascsak RJ, Kozlowski PB, Wisniewski HM. Characteristics of scrapie isolates derived from hay mites. J. Neurovirol. 2000;6:137-44.
Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996;383:685-90.
Collinge, J. Variant Creutzfeld-Javob disease. Lancet 1999;354:317 - 23.
Fischer M B, Roeckl C, Parizek P, Schwarz H.P, Aguzzi A. Binding of diseaseassociated prion protein to plasminogen. Nature 2000;408:479-83.
Hill A.F, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P. The same prion strain causes vCJD and BSE. Nature 1997;44850
Huang Z, Prusiner SB, Cohen FE. Structures of prion proteins and conformational models for prion diseases. In : Prusiner S.B. (ed), Prions, Prions, Prions. Springer-Verlag, Berlin Heidelberg New York, 1996:4965.
Lasmezas CI, Deslys JP, Demaimay R, Adjou KT, Lamoury F, Dormont D, Robain O, Ironside J, Hauw JJ. BSE transmission to macaques. Nature 1996;381:743-4.
Liemann S, Glockshuber R. Transmissible spongiform encephalopathies. "Breakthrough and Views". Biochem. Biophys. Res. Comm. 1998;250:187-93.
Maissen M, Roeckl C, Glatzel M, Goldmann W, Aguzzi A. Plasminogen binds to disease-associated prion protein of multiple species. Lancet 2001;357:2026-8.
Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216:136-44.
Prusiner SB. Prions. Proc. Nat. Sci. USA 1998; 95:13363-83.
Saborio GP, Permanne B, Soto C. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 2001;411:810-13.
Schmerr MJ, Jenny AL, Bulgin MS, Miller JM, Hamir AN, Cutlip RC, Goodwin KR. Use of capillary electrophoresis and fluorescent labeled peptides to detect the abnormal prion protein in the blood of animals
that are infected with a transmissible spongiform encephalopathy. J. Chromatogr. A. 1999;853:207-14.
Scientific Steering Committee. Final Opinion on the Geographical Risk of Bovine Spongiform Encephalopathy (GBR). date: 6/july/2000 website:http://europa.eu.int/comm/food/fs/sc/ssc/outcome_en.html#
opinions)
Scott MR, Will R, Ironside J, Nguyen HO, Tremblay P, DeArmond SJ, Prusiner SB. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc. Natl. Acad. Sci. U S A. 1999 Dec 21;96(26):15137-42.
Shaked GM, Shaked Y, Kariva Z, Halimi M, Avraham I, Gabizon R. A protease resistant isoform is present in urine of animals and humans affected with prion diseases. J. Biol. Chem. 2001 (in press).
Wells GA, Hawkins SA, Green RB, Austin AR, Dexter I, Spencer YI, Chaplin MJ, Stack MJ, Dawson M. Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet Rec. 1998;142:103-6.
Wilesmith JW, Ryan JB, Atkinson MJ. Bovine spongiform encephalopathy: epidemiological studies on the origin. Vet. Rec. 1991;128:199-203.
Wilesmith JW, Ryan JB. Absence of BSE in the offspring of pedigree suckler cows affected by BSE in Great Britain. Vet. Rec. 1997;141:250-1.
Wilesmith JW, Wells A, Ryan JB, Gavier-Widen D, Simmons MM. A cohort study to examine maternally-associated risk factors for bovine spongiform encephalopathy. Vet. Rec.1997;141:239-43.
Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921-5.
Wisniewski HM, Sigurdarson S, Rubenstein R, Kascsak RJ, Carp RI. Mites as vectors for scrapie. Lancet 1996;347:1114.
