Article
The Lymphangioleiomyomatosis Lung Cell and Its Human Cell Models.
American journal of respiratory cell and molecular biology - 1 Jun 2018
Steagall Wendy K, Pacheco-Rodriguez Gustavo, Darling Thomas N, Torre Olga, Harari Sergio, Moss Joel
Abstract excerpt
Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, affecting lungs, kidneys, and lymphatics. It is caused by the proliferation of abnormal smooth muscle-like LAM cells, with mutations and loss of heterozygosity in the TSC1 or, more frequently, TSC2 genes. Isolated pulmonary LAM cells have been difficult to maintain in culture, and most studies of LAM lung cells involve mixtures of TSC2 wild-type...
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