Modern view on prion diseases of the nervous system
https://doi.org/10.30629/2658-7947-2025-30-6-82-90
Abstract
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).
Keywords
prion diseases,
transmissible spongiform encephalopathy,
neurodegenerative diseases,
PrP protein,
Creutzfeldt-Jakob disease
Supporting agencies: The study had no sponsorship
About the Authors
N. V. Selyanina
Ye.A. Vagner Perm State Medical University
Russian Federation
Russian Federation
Perm
M. A. Boytsova
Ye.A. Vagner Perm State Medical University
Russian Federation
Russian Federation
Perm
Yu. V. Karakulova
Ye.A. Vagner Perm State Medical University
Russian Federation
Russian Federation
Perm
T. V. Baidina
Ye.A. Vagner Perm State Medical University
Russian Federation
Russian Federation
Perm
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Selyanina N.V., Boytsova M.A., Karakulova Yu.V., Baidina T.V. Modern view on prion diseases of the nervous system. Russian neurological journal. 2025;30(6):82-90. (In Russ.) https://doi.org/10.30629/2658-7947-2025-30-6-82-90
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