Author/Editor		Jung, Mirko
Title		Creutzfeldt-Jakobova bolezen (CJB) in bovina spongiformna encefalopatija
Translated title		Creutzfeldt-Jakob disease and bovine spongiform encephalopathy
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 66, št. 9
Publication year		1997
Volume		str. 477-85
Language		slo
Abstract		Transmissible spongiform encephalopathies (TSE) are neurodegenerative diseases, that affect human and animals. They are characterized by a spongiform degeneration in the central nervous system (cellular vacuolization and status spongiosus), astrocytic gliosis and neuronal loss; all are associated with the accumulation in affected brains of an abnormal isoform of the normal cellular protein. These diseases are transmissible to experimental animals by inoculation or by ingestion in food. The incubation period in both human and animal disorders is prolonged for years and the outcome is always fatal. Causative agents are resistant to standard decontamination procedures, that inactivate conventional microorganisms. Genetic susceptibility of the host is also relevant in at least some of these diseases. Human TSE include Creutzfeldt-Jakob (hereditary-familiasl form, associated with punctual mutations or insertions in the responsible protein gene; iatrogenic and sporadic form of unknown origin). A new variant of the disease (vCJD) was described in 1996. Known are also Gerstmann-Straeussler-Scheinker syndrome, fatal familial insomnia, kuru and some others, not yet satisfactorily classified diseases. Bovine spongiform encephalopathy (BSE) and scrapie are the most common animal TSE. The molecular nature of these agents is unclear. Mostly discussed hypothesis describes a conformational change in the host protein known as 'prion protein', without involvement of any genetic material. These observations base on the inability to destroy infectivity in purified preparations by methods known to inactivate nucleic acids; the efforts to detect agent-specific nucleic acid were also unsuccesful. (Abstract truncated at 2000 characters.)
Summary		Prenosljive spongiformne encefalopatije (SE) so degenerativne bolezni osrednjega živčevja ljudi in živali. Njihova značilnost so spongiformne degenerativne spremembe osrednjega živčevja (vakuolizacija celic in status spongiozus), astrocitna glioza in propadanje nevronov. V prizadetih možganih se nabira nenormalna amiloidna beljakovina. Bolezni se prenašajo na poskusne živali z inokulacijo ali z uživanjem okužene hrane. Inkubacijska doba za živali in pri ljudeh je zelo dolga in traja do nekaj let; bolezen se vedno fatalno konča. Povzročitelji so odporni na postopke, ki običajne mikroorganizme inaktivirajo. Vsaj za nekatere od teh bolezni je pomembna tudi genetska dovzetnost gostitelja. Med SE pri človeku poznamo bolezen Creutzfeldt-Jakob (CJB) (hereditarno-familiarna oblika, z avtosomno-dominantnim dedovanjem in točkastimi mutacijami ali insercijami na odgovornem genu). Poznani sta še iatrogena oblika in sporadična - neznanega izvora. Nova oblika CJB je bila opisana leta 1996. Poznamo tudi sindrom Gerstmann-Strausler-Scheinker, fatalno familialno insomnijo, kuru in nekatere druge, še ne dovolj pojasnjene bolezni. Bovina spongiformna encefalopatija (BSA) in scrapie (praskavica) sta med živalmi najpogostejši SE. Molekularna narava povzročiteljev ni povsem jasna. Najbolj znana hipoteza opisuje konformacijske spremembe v gostiteljevi beljakovini, poznani kot "prionski beljakovini", ne da bi pri tem bil prizadet genetski material. Ta opazovanja temeljijo na nesposobnosti, da bi uničili infektivnost prečiščenih pripravkov z metodami, za katere vemo, da inaktivirajo nukleinske kisline. Tudi napori, da bi našli za agens specifično nukleinsko kislino, so bili zaman. Nekateri avtorji menijo, da obstaja agensov genom z zaščitenimi nukleinskimi kislinami. (izvleček prekinjen pri 2000 znakih.)
Descriptors		ENCEPHALOPATHY, BOVINE SPONGIFORM CREUTZFELDT-JAKOB SYNDROME DISEASE TRANSMISSION