@article{dd9fde50540c4c3dae8c405ac06b9f2f,
title = "Polycystin-1, the gene product of PKD1, induces resistance to apoptosis and spontaneous tubulogenesis in MDCK cells",
abstract = "The major form of autosomal dominant polycystic kidney disease (ADPKD) results from mutation of a gene (PKD1) of unknown function that is essential for the later stages of renal tubular differentiation. In this report, we describe a novel cell culture system for studying how PKD1 regulates this process. We show that expression of human PKD1 in MDCK cells slows their growth and protects them from programmed cell death. MDCK cells expressing PKD1 also spontaneously form branching tubules while control cells form simple cysts. Increased cell proliferation and apoptosis have been implicated in the pathogenesis of cystic diseases. Our study suggests that PKD1 may function to regulate both pathways, allowing cells to enter a differentiation pathway that results in tubule formation.",
author = "Alessandra Boletta and Feng Qian and Onuchic, {Luiz F.} and Bhunia, {Anil K.} and Bunyong Phakdeekitcharoen and Kazushige Hanaoka and William Guggino and Lucia Monaco and Germino, {Gregory G.}",
note = "Funding Information: A. B. was supported by AHA and the NKF of Maryland. This work was supported by the PKRF, the NIH (DK48006 and DK51042), and Italian Telethon A.112. The authors are grateful to A. Bragonzi and M. Cortese for establishing MDCKtTA cell lines and adapting the expression vectors to the in vitro amplification system, to S. Dahl and K.B. Piontek for critical and helpful discussions, B. Ballermann for use of her equipment, and C. Cooke and the microscope facility at JHU for performing the EM studies. G. G. G. is the Blum Scholar of the JHU School of Medicine. The investigators are members of the Johns Hopkins PKD Center of Excellence (NIH P50-DK57325).",
year = "2000",
doi = "10.1016/S1097-2765(00)00123-4",
language = "English (US)",
volume = "6",
pages = "1267--1273",
journal = "Molecular Cell",
issn = "1097-2765",
publisher = "Cell Press",
number = "5",
}