a micro rna processing defect in rapidly progressing idiopathic pulmonary fibrosis微rna加工缺陷在特发性肺纤维化进展迅速.pdfVIP

A Micro RNA Processing Defect in Rapidly Progressing Idiopathic Pulmonary Fibrosis 1 2 2 2 2 1 Sameer R. Oak , Lynne Murray , Athula Herath , Matthew Sleeman , Ian Anderson , Amrita D. Joshi , 1 3 3 4 3 Ana Lucia Coelho , Kevin R. Flaherty , Galen B. Toews , Darryl Knight , Fernando J. Martinez , Cory M. Hogaboam1* 1 Department of Pathology, University of Michigan Medical School, Ann Arbor, Michigan, United States of America, 2 MedImmune, Cambridge, United Kingdom, 3 Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, United States of America, 4 Department of Anesthesiology, Pharmacology and Therapeutics, UBC James Hogg Research Centre of the Heart+Lung Institute, University of British Columbia, Vancouver, British Columbia, Canada Abstract Background: Idiopathic pulmonary fibrosis exhibits differential progression from the time of diagnosis but the molecular basis for varying progression rates is poorly understood. The aim of the present study was to ascertain whether differential miRNA expression might provide one explanation for rapidly versus slowly progressing forms of IPF. Methodology and Principal Findings: miRNA and mRNA were isolated from surgical lung biopsies from IPF patients with a clinically documented rapid or slow course of disease over the first year after diagnosis. A quantitative PCR miRNA array containing 88 of the most abundant miRNA in the human genome was used to profile lung biopsies from 9 patients with rapidly progressing IPF, 6 patients with slowly progressing IPF