Genes & CellsGenes & CellsГены и КлеткиGenes and Cells2313-18292500-2562Human Stem Cells Institute12196110.23868/202104003ArticlesСтатьиReview ArticleCellular and molecular mechanisms of pulmonary malformationsКлеточные и молекулярные механизмы развития пороков легкихBlinovaS. AБлиноваС. АOripovF. SОриповФ. СKhamidovaF. MХамидоваФ. МSamarkand State Medical InstituteСамаркандский государственный медицинский институт Министерства здравоохранения Республики Узбекистан15032021161VOL 16, NO1 (2021)ТОМ 16, №1 (2021)242816012023Copyright ©; 2021, Eco-VectorCopyright ©; 2021, Эко-Вектор2021Eco-VectorЭко-Векторhttps://genescells.ru/2313-1829/article/view/121961

Until now, the cellular and molecular mechanisms of the development of lung defects remain a poorly studied area of pulmonology. In the occurrence of anomalies in the airways of the lungs, a change in the expression of proteins that control early lung morphogenesis in normal conditions (proteins FGF, TGF, SHH, WNT) was established. Along with this, bronchial markers and markers of type 2 alveolocytes play a certain role in the occurrence of lung defects. A number of congenital malformations are caused by improper formation of the airways, which may be associated with the influence of various soluble factors, receptors, transcription factors and microRNAs. The possible role of the pulmonary neuroendocrine system (apudocytes and neuroepithelial bodies) in the pathogenesis and pathobiology of childhood lung diseases, including congenital lung diseases, is discussed.

До настоящего времени молекулярные и клеточные механизмы развития пороков легких остаются малоизученной областью эмбриологии и пульмонологии. В возникновении аномалий воздухопроводящих путей легких установлена измененная экспрессия белков, контролирующих ранний морфогенез легкого в норме (белки FGF, TGF, SHh, Wnt). Наряду с этим в возникновении пороков легких определенную роль играют бронхиальные маркеры и маркеры альвеолоцитов 2 типа. Ряд врожденных пороков вызван неправильным формированием дыхательных путей, что может быть связано с влиянием различных растворимых факторов, рецепторов, транскрипционных факторов и микроРНК. Обсуждается возможная роль легочной нейроэндокринной системы (апудоцитов и нейроэпителиальных телец) в патогенезе и патобиологии детских заболеваний легких, включая врожденные заболевания легких.

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