The cells comprising pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipomas (AMLs) are heterogeneous, with variable mixtures of cells exhibiting differentiation towards smooth muscle, melanocytic, and lymphatic or blood endothelial cells. 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

Lymphangioleiomyomatosis and TSC2-/- cells / T.N. Darling, G. Pacheco Rodriguez, A. Gorio, E. Lesma, C. Walker, J. Moss. - In: LYMPHATIC RESEARCH AND BIOLOGY. - ISSN 1539-6851. - 8:1(2010 Mar), pp. 59-69. [10.1089/lrb.2009.0031]

Lymphangioleiomyomatosis and TSC2-/- cells

A. Gorio;E. Lesma;
2010

Abstract

The cells comprising pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipomas (AMLs) are heterogeneous, with variable mixtures of cells exhibiting differentiation towards smooth muscle, melanocytic, and lymphatic or blood endothelial cells. 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
Settore BIO/14 - Farmacologia
mar-2010
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/141386
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