Air Space Distension Precedes Spontaneous Fibrotic Remodeling and Impaired Cholesterol Metabolism in the Absence of Surfactant Protein C

What is it about?

The mutation of surfactant protein C is found in cases of familial Idiopathic Pulmonary Fibrosis (IPF). We investigated the events and changes happening in the lungs of mice lacking this protein over their life time. We can now conclude that through time, first the lung is partially collapsed (or closed) and the remained open areas are over expanded. Later in life, wounds appear in the lung permanently closing air-exchange areas. In addition, the lack of the protein induces also abnormal lipid homeostasis (synthesis and degradation). This leads to cholesterol crystals accumulating in alveolar macrophages, cells that are critical for the proper immune defence of the lung.

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