학술논문
PCSK6 and Survival in Idiopathic Pulmonary Fibrosis
Document Type
article
Author
Oldham, Justin M; Allen, Richard J; Lorenzo-Salazar, Jose M; Molyneaux, Philip L; Ma, Shwu-Fan; Joseph, Chitra; Kim, John S; Guillen-Guio, Beatriz; Hernández-Beeftink, Tamara; Kropski, Jonathan A; Huang, Yong; Lee, Cathryn T; Adegunsoye, Ayodeji; Pugashetti, Janelle Vu; Linderholm, Angela L; Vo, Vivian; Strek, Mary E; Jou, Jonathan; Muñoz-Barrera, Adrian; Rubio-Rodriguez, Luis A; Hubbard, Richard; Hirani, Nik; Whyte, Moira KB; Hart, Simon; Nicholson, Andrew G; Lancaster, Lisa; Parfrey, Helen; Rassl, Doris; Wallace, William; Valenzi, Eleanor; Zhang, Yingze; Mychaleckyj, Josyf; Stockwell, Amy; Kaminski, Naftali; Wolters, Paul J; Molina-Molina, Maria; Banovich, Nicholas E; Fahy, William A; Martinez, Fernando J; Hall, Ian P; Tobin, Martin D; Maher, Toby M; Blackwell, Timothy S; Yaspan, Brian L; Jenkins, R Gisli; Flores, Carlos; Wain, Louise V; Noth, Imre
Source
American Journal of Respiratory and Critical Care Medicine. 207(11)
Subject
Language
Abstract
Rationale: Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by limited treatment options and high mortality. A better understanding of the molecular drivers of IPF progression is needed. Objectives: To identify and validate molecular determinants of IPF survival. Methods: A staged genome-wide association study was performed using paired genomic and survival data. Stage I cases were drawn from centers across the United States and Europe and stage II cases from Vanderbilt University. Cox proportional hazards regression was used to identify gene variants associated with differential transplantation-free survival (TFS). Stage I variants with nominal significance (P