Review

ATP-binding cassette (ABC) transporters in normal and pathological lung

Margaretha van der Deen¹ , Elisabeth GE de Vries¹ , Wim Timens³ , Rik J Scheper⁴ , Hetty Timmer-Bosscha¹ and Dirkje S Postma²

¹  University Medical Center Groningen, Department of Internal Medicine, Medical Oncology, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands

²  Department of Pulmonary Medicine, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands

³  Department of Pathology and Laboratory Medicine, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands

⁴  Free University, Department of Pathology, Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands

author email corresponding author email

Respiratory Research 2005, 6:59doi:10.1186/1465-9921-6-59

Published:	20 June 2005

Abstract

ATP-binding cassette (ABC) transporters are a family of transmembrane proteins that can transport a wide variety of substrates across biological membranes in an energy-dependent manner. Many ABC transporters such as P-glycoprotein (P-gp), multidrug resistance-associated protein 1 (MRP1) and breast cancer resistance protein (BCRP) are highly expressed in bronchial epithelium. This review aims to give new insights in the possible functions of ABC molecules in the lung in view of their expression in different cell types. Furthermore, their role in protection against noxious compounds, e.g. air pollutants and cigarette smoke components, will be discussed as well as the (mal)function in normal and pathological lung. Several pulmonary drugs are substrates for ABC transporters and therefore, the delivery of these drugs to the site of action may be highly dependent on the presence and activity of many ABC transporters in several cell types. Three ABC transporters are known to play an important role in lung functioning. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene can cause cystic fibrosis, and mutations in ABCA1 and ABCA3 are responsible for respectively Tangier disease and fatal surfactant deficiency. The role of altered function of ABC transporters in highly prevalent pulmonary diseases such as asthma or chronic obstructive pulmonary disease (COPD) have hardly been investigated so far. We especially focused on polymorphisms, knock-out mice models and in vitro results of pulmonary research. Insight in the function of ABC transporters in the lung may open new ways to facilitate treatment of lung diseases.