r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2025-30-6-82-90r-n-j-780Research ArticleЛЕКЦИЯLECTUREСовременный взгляд на прионные заболевания нервной системыModern view on prion diseases of the nervous systemhttps://orcid.org/0000-0002-2317-7808СелянинаН. В.SelyaninaN. V.

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Perm

nselyanina@mail.ruhttps://orcid.org/0009-0003-9347-6509БойцоваМ. А.BoytsovaM. A.

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Perm

777mascha2002@mail.ruhttps://orcid.org/0000-0002-7536-2060КаракуловаЮ. В.KarakulovaYu. V.

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Perm

julia.karakulova@mail.ruhttps://orcid.org/0000-0002-5114-0463БайдинаТ. В.BaidinaT. V.

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Perm

tatiana_baidina@mail.ruФГБОУ ВО «Пермский государственный медицинский университет им. академика Е.А. Вагнера» Минздрава РоссииРоссияYe.A. Vagner Perm State Medical UniversityRussian Federation2025120220263068290Copyright © Селянина Н.В., Бойцова М.А., Каракулова Ю.В., Байдина Т.В., 20262026Селянина Н.В., Бойцова М.А., Каракулова Ю.В., Байдина Т.В.Selyanina N.V., Boytsova M.A., Karakulova Y.V., Baidina T.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/780

   Прионные заболевания (ПЗ) — редкие нейродегенеративные расстройства, вызываемые аномальными формами белка PrP, способными индуцировать конформационные изменения нормальных белков и приводить к прогрессирующей дегенерации нервной ткани. Эти болезни отличаются длительным инкубационным периодом и высокой летальностью. Важность изучения прионных заболеваний обусловлена необходимостью разработки методов диагностики и профилактики, а также поиска эффективных терапевтических средств. Представлены сведения о механизмах поражения нервной системы, ключевых формах ПЗ (болезнь Крейтцфельдта–Якоба, синдром Герстманна–Штраусслера–Шейнкера, фатальная семейная бессонница и другие), приведены современные методы диагностики с указанием их чувствительности и специфичности, а также рассмотрены перспективные направления терапии (антисмысловые олигонуклеотиды, антитела, ингибиторы агрегации, активация аутофагии, генная терапия).

   Prion diseases (PDs) are rare neurodegenerative disorders caused by abnormal forms of the PrP protein, which can induce conformational changes in normal proteins and lead to progressive degeneration of nerve tissue. These diseases are characterized by a long incubation period and high mortality. The importance of studying PDs stems from the need to develop diagnostic and preventive methods, as well as the search for eff ective therapeutic agents. The article presents information on the mechanisms of damage to the nervous system, on the key forms of PDs (Creutzfeldt–Jakob disease, Gerstmann–Straussler–Scheinker syndrome, fatal familial insomnia, and others), presents modern diagnostic methods with an indication of their sensitivity and specifi city, and also considers promising areas of therapy (antisense oligonucleotides, antibodies, aggregation inhibitors, activation of autophagy, gene therapy).

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The authors declare that there are no conflicts of interest present.