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Mechanisms of Pulmonary Lesions in TSC LAM

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Technical Report,01 May 2017,30 Apr 2018

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Childrens Hospital Los Angeles Los Angeles United States

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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 first year work, we have obtained approvals from both IACUC and ACURO for our animal protocol. We then generated lung mesenchyme-specific Tsc2 mice and harvested the lung tissues from different ages including neonates. Although the sample numbers were relatively small, initial analysis suggested that Tsc2 deletion in lung mesenchyme negatively affect lung alveolar growth during postnatal lung development. The Tsc2-null lung mesenchymal stem cells isolated from this stage showed reduced proliferation comparing to the wild type control. Moreover, the adult mice with Tsc2 genetic deletion induced from embryonic stage E6.5 to E14.5 developed pulmonary nodules 3 out of 3 in females and 1 out of 2 in males. Further analyses and characterization will be performed with additional numbers of lung samples in the second year.

Subject Categories:

  • Medicine and Medical Research
  • Genetic Engineering and Molecular Biology

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