Extracellular matrix microenvironment contributes actively to pulmonary fibrosis

Area of Science:

• Pulmonary Medicine
• Cell Biology
• Biochemistry

Background:

• Idiopathic pulmonary fibrosis (IPF) is a chronic, fatal lung disease of unknown cause.
• IPF is characterized by fibroblast accumulation and extensive extracellular matrix (ECM) deposition.
• The fibrotic lung microenvironment plays a critical role in disease pathogenesis.

Purpose of the Study:

• To describe the contribution of the altered, profibrotic extracellular matrix (ECM) microenvironment to pulmonary fibrosis.
• To explain how the fibrotic ECM changes cell behavior and drives disease progression.
• To highlight the ECM as a potential therapeutic target in pulmonary fibrosis.

Main Methods:

• Review of in-vivo and ex-vivo experimental studies.
• Analysis of the biochemical and biomechanical properties of the fibrotic ECM.
• Examination of growth factor storage within the fibrotic ECM.

Main Results:

• The abnormal ECM in fibrotic lungs alters epithelial and mesenchymal cell behavior.
• The profibrotic ECM microenvironment possesses altered biochemical and biomechanical properties.
• The fibrotic ECM can drive progressive fibrogenesis without further initiating triggers.
• The fibrotic ECM has a complex, active role in lung fibrosis.

Conclusions:

• The fibrotic ECM microenvironment is an active contributor to pulmonary fibrosis development and progression.
• Targeting the profibrotic ECM represents a promising therapeutic strategy for lung fibrosis.