Article
Characterization of cells cultured from chylous effusion from a patient with sporadic lymphangioleiomyomatosis.
Anticancer research - 1 Jun 2015
Grzegorek Irmina, Zuba-Surma Ewa, Chabowski Mariusz, Janczak Dariusz, Szuba Andrzej, Dziegiel Piotr
Abstract excerpt
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a progressive, rare interstitial lung disease that almost exclusively affects women. It is caused by a mutation in one of the tuberous sclerosis genes, TSC1 or TSC2, and constitutive activation of the mammalian target of rapamycin (mTOR) pathway in smooth muscle-like cells (LAM cells). The heightened proliferation and accumulation of LAM cells leads to the destruction...
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