Growth and/or Recruitment of a Novel Cell Population with Neural Crest Origin in Lung Fibrosis
Technical Report,01 Jun 2018,31 May 2019
Childrens Hospital Los Angeles Los Angeles United States
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Pulmonary fibrosis is a debilitating disease characterized by progressive scarring of the lung, which destroys normal lung structure and leads to respiratory failure and death. Abnormally increased fibroblastsmyofibroblasts is one of the key pathological changes in lung fibrosis. The origins of these abnormal lung fibroblastsmyofibroblasts are highly heterogeneous. In this project, we plan to determine whether abnormal growth andor recruitment of a neural crest derived mesenchymal cell population contribute to lung fibrosis. In the past year, we have generated a transgenic reporter mouse line in which neural crest-derived cells were genetically labeled. In normal situation, neural crest-derived cells were detected as nerve fibers adjacent to airway smooth muscles. In contrast, in some bleomycin-induced fibrosis lungs, clusters of neural crest derived cells were detected. This abnormal cellular phenotype varied, and the potential factors affecting this change are currently under investigation. In addition, circulating neural crest-derived mesenchymal progenitor cells were detected in one of five fibrosis mice by peripheral blood mononuclear cell isolation and selective culture. These cultured cells, which were negative for epithelium-marker, were MSC-like progenitors and able to differentiate to other type cells.
- Medicine and Medical Research