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Growth and/or Recruitment of a Novel Cell Population with Neural Crest Origin in Lung Fibrosis
[Technical Report, Final Report]
CHILDREN'S HOSPITAL LOS ANGELES CA
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Pulmonary fibrosis is a debilitating disease characterized by progressive scarring of the lung, which destroys normal lungstructure and leads to respiratory failure and death Abnormally increased fibroblastsmyofibroblasts is one of the key pathologicalchanges 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 populationcontribute to lung fibrosis. In the past year, we have generated a transgenic reporter mouse line in which neural crest-derivedcells were genetically labeled. In normal situation, neural crest-derived cells were detected as nerve fibers adjacent to airwaysmooth muscles. In contrast, in some bleomycin-induced fibrosis lungs, clusters of neural crest derived cells were detected. Thisabnormal 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 a few fibrosis mice by peripheral bloodmononuclear cell isolation and selective culture. These cultured cells, which were negative for epithelium-marker, were MSClike progenitors and able to differentiate to other type cells.
[A, Approved For Public Release]