Chen T, Sun M, Zhou G. Von Hippel-Lindau protein and respiratory diseases. World J Respirol 2013; 3(3): 48-56 [DOI: 10.5320/wjr.v3.i3.48]
Corresponding Author of This Article
Guofei Zhou, PhD, Department of Pediatrics, University of Illinois at Chicago, University of Illinois Hospital and Health Sciences System, 840 S. Wood Street, M/C 856, Ste 1206, Chicago, IL 60612, United States. guofei@uic.edu
Research Domain of This Article
Respiratory System
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Respirol. Nov 28, 2013; 3(3): 48-56 Published online Nov 28, 2013. doi: 10.5320/wjr.v3.i3.48
Von Hippel-Lindau protein and respiratory diseases
Tianji Chen, Miranda Sun, Guofei Zhou
Tianji Chen, Miranda Sun, Guofei Zhou, Department of Pediatrics, University of Illinois at Chicago, University of Illinois Hospital and Health Sciences System, Chicago, IL 60612, United States
Author contributions: Zhou G conceived the study; Chen T, Sun M, and Zhou G wrote the manuscript.
Supported by Grants from Pulmonary Hypertension Association and American Lung Association to Dr. Guofei Zhou
Correspondence to: Guofei Zhou, PhD, Department of Pediatrics, University of Illinois at Chicago, University of Illinois Hospital and Health Sciences System, 840 S. Wood Street, M/C 856, Ste 1206, Chicago, IL 60612, United States. guofei@uic.edu
Telephone: +1-312-3550073 Fax: +1-312-9968204
Received: June 27, 2013 Revised: July 9, 2013 Accepted: July 17, 2013 Published online: November 28, 2013
Core Tip
Core tip: Although von Hippel-Lindau protein (pVHL) was first identified as a tumor suppressor and is best known as a component of ubiquitin protein ligase for the proteasomal degradation of hypoxia inducible factor (HIF)-α, recent studies suggest that pVHL contributes to many lung diseases in both HIF-dependent and HIF-independent pathways. Loss of pVHL promotes pulmonary arterial hypertension via activation of HIF. In lung cancer, loss of pVHL promotes epithelial-mesenchymal transition and cancer migration and invasion while decreasing cell proliferation and colonization. pVHL also induces fibronectin/integrin α5β1/focal adhesion kinase signaling to facilitate fibrogenesis. pVHL mediates Na-K-ATPase degradation to cause decreased edema clearance during hypoxia.
