Wykrywanie białka prionu w płynach ustrojowych – nowe perspektywy w diagnostyce choroby Creutzfeldta-Jakoba

• Authors: Ewa Golańska , Paweł P. Liberski

Title variants

EN

Detection of prion protein in body fluids: new perspectives in Creutzfeldt-Jakob disease diagnostics

• Languages of publication: PL

Abstracts

PL

Choroba Creutzfeldta-Jakoba (Creutzfeldt-Jakob disease, CJD) należy do grupy chorób wywołanych przez priony, których diagnostyka nastręcza trudności ze względu na brak nieinwazyjnych metod umożliwiających przyżyciowe definitywne rozpoznanie. Rutynowe badania laboratoryjne wspomagające diagnostykę polegają na oznaczaniu obecności białka 14-3-3 w płynie mózgowo-rdzeniowym. Białko to jest jednak jedynie nieswoistym markerem rozpadu neuronów. Również badania obrazowe oraz EEG są niewystarczające do pewnego potwierdzenia rozpoznania. Obecnie duże nadzieje wiąże się z rozwojem czułych i swoistych metod wykrywania śladowych ilości nieprawidłowych konformerów białka PrPSc – markera jednoznacznie związanego z chorobami wywołanymi przez priony – znajdujących się w płynie mózgowo-rdzeniowym lub krwi chorych z CJD. W artykule omówiono opracowane w ostatnich latach metody, w których wykorzystuje się zdolność białka prionu do indukowania przekształcenia struktury przestrzennej cząsteczek PrPc w patologiczną izoformę PrPSc. Największe nadzieje na praktyczne zastosowanie budzi obecnie metoda konwersji indukowanej przez wytrząsanie (quaking-induced conversion, QuIC), oparta na amplifikacji nieprawidłowych konformerów białka prionu w warunkach in vitro z zastosowaniem oczyszczonego zrekombinowanego białka PrPc jako substratu. Omówiono zasadę metody, wyniki najnowszych badań nad jej optymalizacją oraz perspektywy wykorzystania w celach diagnostycznych i naukowych.

EN

The diagnostics of prion diseases, including Creutzfeldt-Jakob disease (CJD), is still challenging as none of currently available tests, including magnetic resonance imaging, electroencephalogram and detection of 14-3-3 protein in cerebrospinal fluid, are sufficient for definite premortem diagnosis. This paper presents sensitive methods based on the ability of PrPSc protein to induce the PrPc to PrPSc conversion in vitro which have been developed within the last few years in order to detect trace amounts of the abnormal prion protein in cerebrospinal fluid or blood. Among those methods, the quaking-induced conversion (QuIC) with the use of purified recombined PrPc protein as a substrate seems to be the most promising. We discuss the current research in optimization of the method as well as the perspectives of its possible applications in diagnostics and scientific investigations.

Keywords

EN

Creutzfeldt-Jakob disease; amplifikacja białka prionu; choroba Creutzfeldta-Jakoba; cykliczna amplifikacjanieprawidłowej struktury białka (PMCA); diagnostic markers; konwersja indukowana przez wytrząsanie (QuIC); markery diagnostyczne; prion protein amplification; protein misfolding cyclic amplification (PMCA); quaking-induced conversion (QuIC)

Discipline

• MEDICINE: MEDICINE

• Publisher: Medical Communications
• Journal: Aktualności Neurologiczne
• Year: 2013
• Volume: 13
• Issue: 3
• Pages: 208–216

Contributors

author

Ewa Golańska

• Zakład Patologii Molekularnej i Neuropatologii, Uniwersytet Medyczny w Łodzi

author

Paweł P. Liberski

• Zakład Patologii Molekularnej i Neuropatologii, Uniwersytet Medyczny w Łodzi, nstytut Nauk o Zdrowiu, Państwowa Wyższa Szkoła Zawodowa w Płocku

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• Document Type: article