The lymphangioleiomyomatosis lung cell and its human cell models

Wendy K. Steagall, Gustavo Pacheco-Rodriguez, Thomas N. Darling, Olga Torre, Sergio Harari, Joel Moss

Research output: Contribution to journalReview article

Abstract

Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, affecting lungs, kidneys, and lymphatics. It is caused by the proliferation of abnormal smoothmuscle-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 and TSC2-null cells. A clonal population of LAM lung cells has not been established, making analysis of the cells challenging. Cell lines have been established from angiomyolipomas, a common manifestation of LAM, and from tumors from patients with TSC. Circulating LAM cells have also been isolated from blood and other body fluids. LAM cells may also be identified in clusters apparently derived from lymphatic vessels. Genetics, patterns of antigen expression, and signaling pathways have been studied in LAM lung tissue and in LAM cell models, although rarely all in the same study. We show here that LAM cells manifest differences in these characteristics, depending on the source investigated, suggesting further studies.

Original languageEnglish
Pages (from-to)678-683
Number of pages6
JournalAmerican Journal of Respiratory Cell and Molecular Biology
Volume58
Issue number6
DOIs
Publication statusPublished - Jun 1 2018

Keywords

  • Loss of heterozygosity
  • Lymphangioleiomyomatosis
  • TSC2
  • Tuberous sclerosis

ASJC Scopus subject areas

  • Molecular Biology
  • Pulmonary and Respiratory Medicine
  • Clinical Biochemistry
  • Cell Biology

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