Mechanisms of Pulmonary lesions in TSC LAM
Technical Report,01 May 2019,30 Apr 2020
CHILDRENS HOSPITAL LOS ANGELES CA LOS ANGELES United States
Pagination or Media Count:
The goal of this project is to generate a lung mesenchyme-specific Tsc2 conditional knockout mouse model, in which cystic and nodular lesions may be developed in the lung. In the past year, we have continued the work on 1 To identify the mechanisms by which abrogation of lung mesenchymal Tsc2 results in defective alveolarization and developmental cystic lung pathology in mice 2 To define lung mesenchymal cell origins and the related mechanisms underlying the LAM-like nodular lesions that spontaneously develop in mesenchyme-specific Tsc2 conditional knockout mice. In particular, we have narrowed down the lung mesenchymal cell lineages to those that are targeted by the Tbx4 driver prior to embryonic day 13.5 and are possibly Pdgfrb.Tsc2 deletion in these cell lineages will give rise to pulmonary nodular lesions in later life. In addition, the Tsc2-null lung mesenchymal stem cells have been isolated and cultured for more than 50 passages. These cells are invasive and capable of anchorage-independent growth in vitro.
- Medicine and Medical Research