Article
Mesenchymal folliculin is required for alveolar development: implications for cystic lung disease in Birt-Hogg-Dubé syndrome.
Thorax - 1 Jun 2020
Chu Ling, Luo Yongfeng, Chen Hui, Miao Qing, Wang Larry, Moats Rex, Wang Tiansheng, Kennedy John C, Henske Elizabeth P, Shi Wei
Abstract excerpt
BACKGROUND: Pulmonary cysts and spontaneous pneumothorax are presented in most patients with Birt-Hogg-Dubé (BHD) syndrome, which is caused by loss of function mutations in the folliculin (FLCN) gene. The pathogenic mechanisms underlying the cystic lung disease in BHD are poorly understood. METHODS: Mesenchymal Flcn was specifically deleted in mice or in cultured lung mesenchymal progenitor cells using a Cre/loxP...
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