Amyloidy i prionoidy – czy jest już czas, żeby się obawiać?

• Authors: Paweł P. Liberski

Title variants

EN

Amyloids and prionoids – is there a time to be afraid?

• Languages of publication: PL

Abstracts

PL

Nadeszła nowa era mikrobiologii, kiedy zrozumieliśmy, że niekonwencjonalne wirusy kuru, choroby Creutzfeldta--Jakoba (CJD) i zespołu [obecnie „choroby”] Gerstmanna-Sträusslera-Scheinkera (GSS), scrapie, encefalopatii gąbczastej bydła (BSE) są infekcyjnymi białkami amyloidowymi i że pasażowalne encefalopatie gąbczaste są amyloidozami mózgu. Ten cytat z wypowiedzi laureata Nagrody Nobla D. Carletona Gajduska znakomicie ilustruje całość zagadnienia. Amyloid to nazwa ogólna określająca włókienkową czwartorzędową strukturę białka. Wszystkie amyloidy, niezależnie od sekwencji aminokwasów tworzących je białek, tworzą się w wyniku reakcji nukleacji/polimeryzacji, w której agregaty (oligomery), składające się z niewielkiej liczby cząsteczek białka (jądro, seed), nukleują cząsteczki białka prekursorowego, co prowadzi do zmiany konformacji przestrzennej w kierunku harmonijki-β. Choroby wywołane przez takie białka nazywa się prionoidami. „Prawdziwe” priony różnią się zasadniczo od wszystkich innych prionoidów − priony są zakaźnie w sensie mikrobiologicznym, szerzą się między osobnikami, wywołując makroepidemie, takie jak kuru, vCJD, BSE i jatrogenne przypadki CJD. W niniejszym artykule zostaną omówione podstawowe prionoidy − choroby Alzheimera i Parkinsona – oraz relacja łącząca je z chorobami prionowymi.

EN

The new era has come to microbiology as we have realized that the unconventional viruses of kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), scrapie, and bovine spongiform encephalopathy (BSE) are infectious amyloid proteins and that these transmissible spongiform dementias are brain amyloidoses. This quotation from a Nobel laureate, D. Carleton Gajdusek, illustrates the best the content of this paper. Amyloid is a generic term, which embraces the fibrillary cross-β-sheet quaternary structure of any protein. All amyloids, irrespective of their amino acid sequences, are formed through nucleation/polymerization reactions in which oligomeric structures (small aggregates) composed of a limited number of a given protein moiety (a seed) nucleates other moieties. As a result, the β-pleated secondary structure predominates. Such proteins are called “prionoids” as opposed to “real” prions, which are infectious, or transmissible, in a microbiological sense; they spread between individuals and cause macro-epidemics, such as kuru, BSE and iatrogenic CJD. In this review, prions and prionoids, and their inter-relatedness, will be discussed.

Keywords

EN

amyloids; amyloidy; nucleation; nukleacja; oligomers; oligomery; prionoids; prionoidy; prions; priony

Discipline

• MEDICINE: MEDICINE

• Publisher: Medical Communications
• Journal: Aktualności Neurologiczne
• Year: 2013
• Volume: 13
• Issue: 3
• Pages: 230–239

Contributors

author

Paweł P. Liberski

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

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