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• W2062236960 endingPage "69" @default.
• W2062236960 startingPage "59" @default.
• W2062236960 abstract "The cells comprising pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipomas (AMLs) are heterogeneous, with variable mixtures of cells exhibiting differentiation towards smooth muscle, fat, and vessels. Cells grown from LAM and AMLs have likewise tended to be heterogeneous. The discovery that LAM and AMLs contain cells with mutations in the TSC1 or TSC2 genes is allowing investigators to discriminate between “two-hit” cells and neighboring cells, providing insights into disease pathogenesis. In rare cases, it has been possible to derive cells from human tumors, including AMLs and TSC skin tumors that are highly enriched for TSC2-/- cells. Cells derived from an Eker rat uterine leiomyoma (ELT3 cells) are Tsc2-null and these have been used in a rodent cell models for LAM. Further improvements in the ability to reliably grow well-characterized TSC2-/- cells from human tumors are critical to developing in vitro and in vivo model systems for studies of LAM pathogenesis and treatment." @default.
• W2062236960 created "2016-06-24" @default.
• W2062236960 creator A5048042168 @default.
• W2062236960 creator A5051898747 @default.
• W2062236960 creator A5053241457 @default.
• W2062236960 creator A5059088289 @default.
• W2062236960 creator A5060545410 @default.
• W2062236960 creator A5072682608 @default.
• W2062236960 date "2010-03-01" @default.
• W2062236960 modified "2023-10-18" @default.
• W2062236960 title "Lymphangioleiomyomatosis and<i>TSC2^-/-</i>Cells" @default.
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