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UDK: 577, 616.89 DOI:10.33920/med-01-2511-08

A transgenic mouse line carrying the P654L (rs139336954) variant in the brain morphogenic protein Plxna3 reveals signs of obsessive-compulsive personality disorder

Karagyaur Maxim Nikolaevich D.Sc. (Biology), Associate Professor, Department of Biochemistry and Regenerative Biomedicine, Senior Researcher, Center for Regenerative Medicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0003-4289-3428
Averina Olga Alexandrovna Ph.D. (Chemistry), Leading Researcher, A.N. Belozersky Institute Of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow, Russia, IRID: 8768387, ORCID: 0000‑0002‑8244‑526X
Bozov Kirill Dmitrievich Research Assistant, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0009-0006-8442-8582
Dzhauari Stalik Stanislavovich Ph.D. (Biology), Tutor, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0001-5480-2322
Priymak Anastasia Vladimirovna Leading Engineer, A.N. Belozersky Institute Of Physico-Chemical Biology, Research Laboratory Assistant, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, IRID: 636121456
Permyakov Oleg Aleksandrovich Engineer 2nd Category, A.N. Belozersky Institute Of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow, Russia, Researcher ID: AAT 7369–2021, IRID: 31972943
Grigorieva Olga Olegovna Ph.D. (Biology), Junior Researcher, A.N. Belozersky Institute Of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow, Russia, IRID: 257639733
Illarionova Maria Evgenievna Research Assistant, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0009-0006-2483-2137
Shkarina Liliia Nikolaevna Researcher, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Education Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0009-0005-1774-3534
Primak Alexandra Leonidovna Research Assistant, Department of Biochemistry and Regenerative Biomedicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0002-1386-5922
Khaybullina Raushana Timurovna student at the Faculty of Fundamental Medicine, Medical Research and Education Institute, Lomonosov Moscow State University, Moscow, Russia
Semina Ekaterina Vladimirovna D.Sc. (Biology), Leading Researcher, Laboratory of Tissue Morphogenesis and Reparation, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0002-3927-9286
Klimovich Polina Sergeevna Ph.D. (Biology), Senior Researcher, Laboratory of Tissue Morphogenesis and Reparation, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0002-8260-5542
Samokhodskaya Larisa Mikhailovna Ph.D. (Medical Sciences), Head of the Laboratory Diagnostics Department, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0001-6734-3989
Sergiev Petr Vladimirovich D.Sc. (Chemistry), Corresponding Member of the Russian Academy of Sciences, Director, A.N. Belozersky Institute Of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow, Russia, IRID: 389331, Researcher ID: D-7493-2012, Scopus Author ID: 7004499861
Popov Vladimir Sergeevich Ph.D. (Biology), Head of the Research Laboratory of Translational Medicine, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0002-5039-7152
Neyfeld Elena Arslanalievna Ph.D. (Medical Sciences), Chief Physician, Mental Health Research Center, Associate Professor, Department of Multidisciplinary Clinical Training, Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0003-2647-1284
Tsygankov Boris Dmitrievich D.Sc. (Medical Sciences), Corresponding Member of the Russian Academy of Sciences, Head of Department, Russian University of Medicine of the Ministry of Healthcare of the Russian Federation, Moscow, Russia, ORCID: 0000-0003-0180-1267
Tkachuk Vsevolod Arsenievich D.Sc. (Biology), Academician of the Russian Academy of Sciences, Director of the Medical Research and Educational Institute, Lomonosov Moscow State University, Moscow, Russia, ORCID: 0000-0002-7492-747X

Genetic technologies open up opportunities for studying the molecular basis of a wide range of hereditary pathologies, including mental disorders. Modeling certain genomic variants in cellular and animal models allows us to establish their functional significance and potential mechanisms of involvement in the disease progression, as well as lays the material basis for the development and testing of promising drugs. Here, in this study, we created the first line of transgenic mice carrying the P654L (rs139336954) mutation in the Plxna3 nerve tissue morphogenesis protein and studied their cognitive and behavioral characteristics. The Plxna3 protein, together with the Nrp1/Nrp2 co-receptor, detects signals from the repellent molecules Sema3a and Sema3f and determines the direction of cell migration and nerve fiber growth, the processes that are crucial for the proper development of the finely tuned nervous system. According to bioinformatic analysis, this mutation alters the structure of the extracellular domain of Plxna3, which could potentially disrupt the reception of Sema3a and/or Sema3f. Studies of the behavior of mice carrying the Plxna3-P654L (rs139336954) mutation have revealed increased anxiety, a high degree of orderliness, and reduced sensitivity to external stimuli, which could potentially be interpreted as symptoms of obsessive-compulsive personality disorder. No significant macroscopic or histological differences in brain structure were detected between the control and experimental groups. In this study, we draw attention for the first time to the genomic variant rs139336954 in the Plxna3 gene as a potential cause of obsessive-compulsive personality disorder and propose a novel transgenic model organism for studying the pathogenesis of this disease.

Обсессивно-компульсивное расстройство (ОКР) или невроз навязчивых состояний – психическое расстройство, характеризующееся повторяющимися навязчивыми мыслями, образами, импульсами или идеями, обычно вызывающими беспокойство или страдание, а также повторяющимися мыслительными и/или поведенческими феноменами [1–3]. Согласно последним данным, распространенность ОКР в популяции может достигать 1–3 % [4, 5]. ОКР ухудшает качество жизни пациентов, значительно нарушает их социальную и трудовую адаптацию, а в ряде случаев приводит к трансформации навязчивых мыслей в бред и значительно увеличивает суицидальный риск [6, 7].

Среди причин развития ОКР выделяют, как генетические факторы, так и факторы окружающей среды. Предполагается, что в основе патогенеза ОКР лежит дисбаланс активности кортико-стриато-таламо-кортикальной системы [8], в частности ее глутаматергический, серотониновый и/или дофаминовый компонент [4, 9]. Близнецовые исследования и исследования семей с ОКР подтвердили наличие существенного наследственного компонента в этиопатогенезе данного заболевания, причем наибольший вклад наследственных факторов был отмечен для форм ОКР с манифестацией в детском возрасте [10]. На сегодняшний день было идентифицировано несколько генов (Hoxb8, Slc1a1, Sapap3, Slitrk5, Spred2), полный нокаут которых у мышей приводит к появлению у мышей паттернов, поведения, соответствующего клиническим проявлениям ОКР [11–13]. Однако какие генетические варианты (даже в указанных генах) ассоциированы с риском развития ОКР, еще только предстоит установить.

Любопытным является тот факт, что по меньшей мере три из названных генов (Hoxb8, Sapap3 и Slitrk5) являются активными участниками процессов развития мозга: участвуют в пролиферации, миграции и дифференцировке нейральных предшественников, выборе направления роста нейритов и созревании синапсов [14, 15]. Это подтверждает важность правильного развития головного мозга для сохранности его психической и когнитивной функции, а также тот факт, что нарушение формирования мозга ввиду несвоевременной экспрессии или дисфункции молекул, обеспечивающих пролиферацию и миграцию нейральных предшественников, их прецизионное позиционирование в ткани мозга и соединение в функциональные нейронные сети, может лежать в основе развития психических заболеваний [16, 17].

For citation:
Karagyaur Maxim Nikolaevich, Averina Olga Alexandrovna, Bozov Kirill Dmitrievich, Dzhauari Stalik Stanislavovich, Priymak Anastasia Vladimirovna, Permyakov Oleg Aleksandrovich, Grigorieva Olga Olegovna, Illarionova Maria Evgenievna, Shkarina Liliia Nikolaevna, Primak Alexandra Leonidovna, Khaybullina Raushana Timurovna, Semina Ekaterina Vladimirovna, Klimovich Polina Sergeevna, Samokhodskaya Larisa Mikhailovna, Sergiev Petr Vladimirovich, Popov Vladimir Sergeevich, Neyfeld Elena Arslanalievna, Tsygankov Boris Dmitrievich, Tkachuk Vsevolod Arsenievich, A transgenic mouse line carrying the P654L (rs139336954) variant in the brain morphogenic protein Plxna3 reveals signs of obsessive-compulsive personality disorder. Bulletin of Neurology, Psychiatry and Neurosurgery. 2025;11.
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