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New Developments in the Pathogenesis of Pulmonary Cysts in Birt-Hogg-Dubé Syndrome.

Seminars in respiratory and critical care medicine - 1 Apr 2020

Kennedy John C, Khabibullin Damir, Boku Yoseph, Shi Wei, Henske Elizabeth P

Abstract excerpt

Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder caused by germline loss-of-function mutations in Folliculin gene (FLCN). BHD is characterized by lower lobe-predominant pulmonary cysts with risk of pneumothorax, benign skin tumors (fibrofolliculomas), and renal cell carcinoma, often of an unusual chromophobe/oncocytic hybrid histology. The FLCN protein functions in multiple signaling and metabolic...

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PMID: 32279295
DOI: 10.1055/s-0040-1708500

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