Болезнь Крейтцфельдта-Якоба: современные аспекты проблемы (обзор литературы)

A. V. Peresedova1, I. A. Zavalishin1
1ФГБУ «Научный центр неврологии» РАМН (Москва), Россия

Аннотация


 

Болезнь Крейтцфельдта-Якоба (БКЯ) относится к прионным болезням человека – группе фатальных нейродегенеративных заболеваний. Согласно этиологической классификации выделяют спорадические (идиопатические), приобретенные и наследственные формы. Возбудителем прионных болезней является инфекционный прионный белок (PrPSc), образующийся в результате конформационных изменений нормального (неинфекционного) клеточного белка PrPC. В статье освещены вопросы молекулярной классификации спорадической БКЯ, фенотипической вариабельности, приведены основные патогенетические механизмы при прионных заболеваниях. Уникальная резистентность прионов к классическим методам обеззараживания и возможность ятрогенной трансмиссии определяет необходимость строгого контроля за соблюдением процедур обеззараживания. В экспериментальных условиях (культура клеток и экспериментальные животные) разрабатываются различные терапевтические подходы при прионных заболеваниях, однако в клинической практике проведены или проводятся лишь несколько исследований.


Ключевые слова

болезнь Крейтцфельдта-Якоба; прион

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Литература

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