ATP-binding cassette (ABC) transporters in normal and pathological lung
<p>Abstract</p> <p>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-ass...
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| Format: | Article |
| Language: | English |
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BMC
2005-06-01
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| Series: | Respiratory Research |
| Online Access: | http://respiratory-research.com/content/6/1/59 |
| _version_ | 1819043042088189952 |
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| author | Timmer-Bosscha Hetty Scheper Rik J Timens Wim de Vries Elisabeth GE van der Deen Margaretha Postma Dirkje S |
| author_facet | Timmer-Bosscha Hetty Scheper Rik J Timens Wim de Vries Elisabeth GE van der Deen Margaretha Postma Dirkje S |
| author_sort | Timmer-Bosscha Hetty |
| collection | DOAJ |
| description | <p>Abstract</p> <p>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 (<it>CFTR</it>) gene can cause cystic fibrosis, and mutations in <it>ABCA1 </it>and <it>ABCA3 </it>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 <it>in vitro </it>results of pulmonary research. Insight in the function of ABC transporters in the lung may open new ways to facilitate treatment of lung diseases.</p> |
| first_indexed | 2024-12-21T09:50:29Z |
| format | Article |
| id | doaj.art-ac8596ed845a442d83d542f44b9955d6 |
| institution | Directory Open Access Journal |
| issn | 1465-9921 |
| language | English |
| last_indexed | 2024-12-21T09:50:29Z |
| publishDate | 2005-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Respiratory Research |
| spelling | doaj.art-ac8596ed845a442d83d542f44b9955d62022-12-21T19:08:12ZengBMCRespiratory Research1465-99212005-06-01615910.1186/1465-9921-6-59ATP-binding cassette (ABC) transporters in normal and pathological lungTimmer-Bosscha HettyScheper Rik JTimens Wimde Vries Elisabeth GEvan der Deen MargarethaPostma Dirkje S<p>Abstract</p> <p>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 (<it>CFTR</it>) gene can cause cystic fibrosis, and mutations in <it>ABCA1 </it>and <it>ABCA3 </it>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 <it>in vitro </it>results of pulmonary research. Insight in the function of ABC transporters in the lung may open new ways to facilitate treatment of lung diseases.</p>http://respiratory-research.com/content/6/1/59 |
| spellingShingle | Timmer-Bosscha Hetty Scheper Rik J Timens Wim de Vries Elisabeth GE van der Deen Margaretha Postma Dirkje S ATP-binding cassette (ABC) transporters in normal and pathological lung Respiratory Research |
| title | ATP-binding cassette (ABC) transporters in normal and pathological lung |
| title_full | ATP-binding cassette (ABC) transporters in normal and pathological lung |
| title_fullStr | ATP-binding cassette (ABC) transporters in normal and pathological lung |
| title_full_unstemmed | ATP-binding cassette (ABC) transporters in normal and pathological lung |
| title_short | ATP-binding cassette (ABC) transporters in normal and pathological lung |
| title_sort | atp binding cassette abc transporters in normal and pathological lung |
| url | http://respiratory-research.com/content/6/1/59 |
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