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Defects in the process of degradation of unneeded cellular proteins underlie many diseases. This article discusses one of the most important systems of removal of abnormal proteins. It describes the process of ubiquitination of proteins for proteasome degradation. It also describes the structure of the 26S and 20S proteasomes and the mechanism of ubiquitin-proteasome system. Proteasome proteolytic system is highly specialized and organized. Protease-proteasome 26S is particularly important for proper cell functioning. It recognizes and degrades marked proteins. Inhibition of proteasome pathway leads to cell cycle arrest and apoptosis.
Efficient degradation of cellular proteins by UPS (the ubiquitin - proteasome system) - is important for signal transduction, transcriptional regulation, response to stress and the activity control of cell receptors.
The development of many diseases has its origin in the dysfunction of the UPS route. This group includes diseases such as cancer, neurodegenerative disorders, immune-mediated diseases and infectious diseases. Development of effective methods for pharmacological intervention in the functioning of this system has become a great challenge. The use of specific, low molecular-weight proteasome inhibitors and enzymes catalyzing the ubiquitination gives hope for new, targeted therapies.
Aleksandra Siek, Agata Makarewicz, Łukasz Łobejko, Anna Gralewska, Joanna Tomaka, Justyna Szymańska-Piekarczyk, Jakub Siembida and Hanna Karakuła Juchnowicz
Introduction: Trichotillomania is a mental disorder characterized by a repetitive and compulsive hair pulling, classified in ICD-10 to a group of habit and impulse disorders, and in the DSM-5 to the group of obsessive-compulsive disorders.
Aim: The aim of the study is to present on the basis of case study:1). the importance of traumatic family experiences in releasing as well as maintaining the symptoms of Trichotillomania, 2). comprehensive medical care, the application of which has resulted in a beneficial therapeutic effect.
Results: In the described case of 16-year-old patient, Trichotillomania was triggered by traumatic events related to lack of support and family stabilization resulting from parental disputes and grandfather’s death, when she was 11 years old. The subsequent years of her life, in spite of the divorce of her parents and their separate residence, abounded in periods of turbulent quarrels between the parents in which she was involved. Each time this type of incident was associated with the recurrence of behavior associated with Trichotil-lomania, the course of which was more severe with the occurrence of self-harm and suicidal thoughts.
Conclusions: 1. In the described case, traumatic events and pathological relations of the immediate family members were not only thetriggering factor, but also maintaining the Trichotillomania symptoms. 2. In accordance with the guidelines of Trichotillomania Learning Center-Scientific Advisory Board (2008), the use of a comprehensive treatment including both the patient - individual psycho-therapy (especially cognitive-behavioral therapy) and pharmacotherapy, as well as her family (family psychotherapy, family mediation, workshops for parents), brought about positive therapeutic effects.
Josef Parnas, Paul Møller, Tilo Kircher, Jørgen Thalbitzer, Lennart Jansson, Peter Handest, Dan Zahavi, Hanna Karakuła-Juchnowicz, Justyna Morylowska-Topolska and Dariusz Juchnowicz
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Paulina Wróbel-Knybel, Michał Flis, Rafał Dubiel and Hanna Karakuła-Juchnowicz
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