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Autor:
Dmitry A. Goncharov, Seung-Beom Hong, Melane L. James, Laura S. Schmidt, Masaya Baba, Andrew J. Gow, W. Marston Linehan, Hua Li, Susan H. Guttentag, Susan S. Margulies, Linda W. Gonzales, Vera P. Krymskaya, Elena A. Goncharova, Victoria Stepanova, Elena N. Atochina-Vasserman
Publikováno v:
Cell Reports, Vol 7, Iss 2, Pp 412-423 (2014)
Cell reports, vol 7, iss 2
Cell reports, vol 7, iss 2
Summary: Spontaneous pneumothoraces due to lung cyst rupture afflict patients with the rare disease Birt-Hogg-Dubé (BHD) syndrome, which is caused by mutations of the tumor suppressor gene folliculin (FLCN). The underlying mechanism of the lung mani