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Familial Cancer (2013) 12:133–140 DOI 10.1007/s10689-012-9528-4 AB STRACTS The Fourth Birt–Hogg–Dube ´ Symposium, Cincinnati, USA, th th 28 –30 March, 2012 Springer Science+Business Media B.V. 2012 Nephrology and Hypertension, University Medical Center Utrecht, FLCN loss causes hematopoietic stem cell homeostasis Utrecht, 3584 CX, The Netherlands, email@example.com; dysregulation and myeloproliferative disease 2 3 University Medical Center Utrecht; Maastricht University Medical Center 1 2 3 Masaya Baba , Hyung-Chan Suh , Hisashi Hasumi , 3 2 3 3 Ciliopathy is a general term applied to diseases that originate from Yukiko Hasumi , Ming Ji , Mara E. Klein , Laura S. Schmidt , 3 2 ciliary dysfunction, which often coincide with renal cyst develop- W. Marston Linehan , Jonathan R. Keller ment. Birt-Hogg-Dube (BHD) syndrome was therefore suspected as a Staff Scientist, Urologic Oncology Branch, National Cancer novel ciliopathy. Indeed, folliculin (FLCN), the BHD gene product, Institute, NIH, Bethesda, MD, 20892, United States, localizes to primary cilia. To study ciliopathies in detail, we set up a firstname.lastname@example.org; National Cancer Institute-Frederick, NIH; renal 3D culture system that physiologically mimic polarized renal National Cancer Institute, NIH tubuli. Upon SiFlcn in this system, several affected processes were identiﬁed; (1) Reduction of ciliation in vitro
Familial Cancer – Springer Journals
Published: Mar 1, 2013
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