sibmedСибирский научный медицинский журналСибирский научный медицинский журнал2410-25122410-2520ИЦиГ СО РАН10.15372/SSMJ20190401sibmed-202Research ArticleОБЗОРЫREVIEWSМОРФОФУНКЦИОНАЛЬНАЯ ХАРАКТЕРИСТИКА МИТОХОНДРИЙ И ИММУНОГИСТОХИМИЧЕСКИЕ ПОДХОДЫ К ИХ ИЗУЧЕНИЮMORPHOFUNCTIONAL CHARACTERISTICS OF MITOCHONDRIA AND IMMUNOHISTOCHEMICAL APPROACHES TO THEIR STUDYБоньЕ. И.BonL. I.

к.б.н., 

Республика Беларусь, 230009, г. Гродно, ул. Горького, 80

candidate of biological sciences

Republic Belarus, 230009, Grodno, Gorkogo str., 80

e_bon@list.ruМаксимовичН. Е.MaksimovichN. Ye.

д.м.н., проф., 

Республика Беларусь, 230009, г. Гродно, ул. Горького, 80

doctor of medical sciences, professor

Republic Belarus, 230009, Grodno, Gorkogo str., 80

mne@grsmu.byГродненский государственный медицинский университетБеларусьGrodno State Medical UniversityBelarus201904092019394511Copyright © Бонь Е.И., Максимович Н.Е., 20192019Бонь Е.И., Максимович Н.Е.Bon L.I., Maksimovich N.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://sibmed.elpub.ru/jour/article/view/202

Нарушением функций митохондрий сопровождается любое заболевание, поэтому дальнейшее исследование функциональных особенностей митохондрий при различных патологиях в клинике и эксперименте, а также поиск новых диагностических маркеров перспективны и актуальны. Цель настоящего обзора – обобщение и систематизация данных литературы о морфофункциональных характеристиках и молекулярных иммуногистохимических маркерах, используемых для оценки функции митохондрий. Наиболее характерной особенностью этих органелл является наличие большого числа ферментов, участвующих в окислительном фосфорилировании и снабжении клетки энергией. Кроме того, митохондрии играют немаловажную роль в хранении и передаче наследственной информации, апоптозе и пластических процессах. Выделяют так называемые митохондриальные болезни, связанные с генетическими, структурными, биохимическими дефектами митохондрий, в том числе приводящими к энергодефициту клеток. Они передаются по женской линии, так как только яйцеклетка содержит митохондрии. Известны наследственные митохондриальные заболевания, связанные с мутациями генов, кодирующих синтез митохондриальных белков, – синдром Барта, синдром Кернса – Сейра, синдром Пирсона и др. Существует ряд молекулярных маркеров, использование которых позволяет детально изучить активность митохондрий при различных экспериментальных воздействиях. Известно около тысячи митохондриальных маркеров, но в настоящем обзоре представлены основные.

Violation of the functions of mitochondria is accompanied by any disease, so further study of the functional characteristics of mitochondria in various pathologies in the clinic and experiment, as well as the search for new diagnostic markers is promising and relevant. The purpose of this review is to summarize and systematize the literature data on morphofunctional characteristics and molecular immunohistochemical markers used to assess the function of mitochondria. The most characteristic feature of these organelles is the presence of a large number of enzymes involved in oxidative phosphorylation and energy supply to the cell. Also, the aim was to allocate so-called mitochondrial diseases associated with genetic, structural, biochemical defects of mitochondria, including those leading to energy deficiency of cells. Mitochondrial diseases are transmitted through the female line, since only the ovum contains mitochondria. Hereditary mitochondrial diseases associated with mutations in the genes encoding the synthesis of mitochondrial proteins – Bart’s syndrome, Kearns – Sayre syndrome, Pearson’s syndrome, and others are known. In addition, mitochondria are involved in the storage and transmission of hereditary information, apoptosis and plastic processes. There are a number of molecular markers, the use of which allows a detailed study of the activity of mitochondria under various experimental effects. The nearly thousand mitochondrial markers are known, but this review encloses the main ones.

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