Epithelial cells in pulmonary fibrosis
Kosunen, Karoliina (2024)
Kosunen, Karoliina
2024
Bioteknologian ja biolääketieteen tekniikan kandidaattiohjelma - Bachelor's Programme in Biotechnology and Biomedical Engineering
Lääketieteen ja terveysteknologian tiedekunta - Faculty of Medicine and Health Technology
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Hyväksymispäivämäärä
2024-05-06
Julkaisun pysyvä osoite on
https://urn.fi/URN:NBN:fi:tuni-202404224002
https://urn.fi/URN:NBN:fi:tuni-202404224002
Tiivistelmä
Pulmonary fibrosis is a pathological condition characterized by the accretion of extracellular matrix in the space between the alveoli. Idiopathic pulmonary fibrosis (IPF) is a form of this condition where the precise etiology is unknown. IPF is an aggressively progressive disease affecting approximately three million people worldwide. Currently, apart from surgical lung transplantation, there are no clinically effective treatments for the disease.
IPF is a molecularly complex disease. Central to the pulmonary fibrotic response in the lung is the abnormal activation of alveolar epithelial cells, which initiates the pathogenesis of the disease. The alveolar epithelium is composed of type 1 (AEC1) and type 2 (AEC2) alveolar epithelial cells responsible for maintaining lung homeostasis. AEC1 cells facilitate gas exchange and ion and fluid balance in the lung, while AEC2 cells are responsible for lung regeneration, being capable of differentiating into type 1 cells. Additionally, type 2 cells secrete surfactant, which prevents lung collapse during exhalation.
During lung injury, the normal function of alveolar epithelial cells is disrupted. The dysfunction progresses through three stages, ultimately leading to fibrotic changes. These changes are manifested by impaired communication between the alveolar epithelium and fibroblasts, epithelial-mesenchymal transition, and progressive accumulation of collagen and extracellular matrix. The fibrotic environment is shaped not only by the response of AEC cells but also by the interactions between the lung microbiome, transmitters, and immune cells.
This literature review aims to present the pathological role of alveolar epithelial cells and their interactions with other cells relevant to IPF. Based on the collected literature, it can be concluded that alveolar epithelial cells, especially type 2 cells, play a significant role in the pathogenesis of IPF. An improved understanding of IPF pathomechanisms is necessary for the development of innovative and effective treatments, a goal supported by usage of various in vitro and in vivo models.
IPF is a molecularly complex disease. Central to the pulmonary fibrotic response in the lung is the abnormal activation of alveolar epithelial cells, which initiates the pathogenesis of the disease. The alveolar epithelium is composed of type 1 (AEC1) and type 2 (AEC2) alveolar epithelial cells responsible for maintaining lung homeostasis. AEC1 cells facilitate gas exchange and ion and fluid balance in the lung, while AEC2 cells are responsible for lung regeneration, being capable of differentiating into type 1 cells. Additionally, type 2 cells secrete surfactant, which prevents lung collapse during exhalation.
During lung injury, the normal function of alveolar epithelial cells is disrupted. The dysfunction progresses through three stages, ultimately leading to fibrotic changes. These changes are manifested by impaired communication between the alveolar epithelium and fibroblasts, epithelial-mesenchymal transition, and progressive accumulation of collagen and extracellular matrix. The fibrotic environment is shaped not only by the response of AEC cells but also by the interactions between the lung microbiome, transmitters, and immune cells.
This literature review aims to present the pathological role of alveolar epithelial cells and their interactions with other cells relevant to IPF. Based on the collected literature, it can be concluded that alveolar epithelial cells, especially type 2 cells, play a significant role in the pathogenesis of IPF. An improved understanding of IPF pathomechanisms is necessary for the development of innovative and effective treatments, a goal supported by usage of various in vitro and in vivo models.
Kokoelmat
- Kandidaatintutkielmat [8709]