Estrogen and the cystic fibrosis gender gap |
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| Affiliation: | 1. Institut für Pharmakologie und Präventive Medizin, Mahlow, Germany;2. Institut für Statistik, Universität Ulm, Ulm, Germany;3. Institut für Biometrie und Epidemiologie, Deutsches Diabetes Zentrum, Leibniz-Zentrum für Diabetesforschung an der Heinrich-Heine Universität, Düsseldorf, Germany;4. Gemeinschaftspraxis Kaltheuner – v. Boxberg, Leverkusen, Germany;5. winDiab GmbH, Düsseldorf, Germany;6. Kinder- und Jugendkrankenhaus “AUF DER BULT”, Hannover, Germany;1. Laboratory of Veterinary Anatomy, College of Veterinary Medicine, China Agricultural University, Beijing 100193, China;2. Laboratory of Veterinary Anatomy, College of Animal Science and Technology, Henan University of Science and Technology, Luoyang 471000, China;3. Yanjing Medical College, Capital Medical University, Beijing 101300, China;4. Department of Environmental Toxicology, University of California, Davis, CA 95616, USA;1. Environmental Protection Agency, 1595 Wynkoop St, Denver, CO, 80202, United States;2. University of Cincinnati, Department of Environmental Health, 160 Panzeca Way, Cincinnati, OH, 45267, United States;3. University of Cincinnati, Department of Internal Medicine, Pulmonary Division, 160 Panzeca Way, Cincinnati, OH 45267, United States;4. SRC Inc., Suite 1380, 999 18th St, Denver, CO, 80202, United States;1. Department of Quality Control, Razi Vaccine and Serum Research Institute, Karadj, (VM), Biotechnology Department (NM, SK) Department of Microbiology (MR), Iran Reference Health Lab, Ministry of Health and Medical Education, and Pathology, Milad National Hospital, Tehran, Departments of Medical Vaccine Research (SMM), Pathology (AH), Razi Vaccine and Serum Research Institute, Karadj, Iran;1. Department of Pharmaceutical Sciences, North Dakota State University, Fargo, ND, USA;2. Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, MN, USA;3. Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA |
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| Abstract: | Cystic fibrosis (CF) is the most frequent inherited disease in Caucasian populations and is due to a defect in the expression or activity of a chloride channel encoded by the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Mutations in this gene affect organs with exocrine functions and the main cause of morbidity and mortality for CF patients is the lung pathology in which the defect in CFTR decreases chloride secretion, lowering the airway surface liquid height and increasing mucus viscosity. The compromised ASL dynamics leads to a favorable environment for bacterial proliferation and sustained inflammation resulting in epithelial lung tissue injury, fibrosis and remodeling. In CF, there exist a difference in lung pathology between men and women that is termed the “CF gender gap”. Recent studies have shown the prominent role of the most potent form of estrogen, 17β-estradiol in exacerbating lung function in CF females and here, we review the role of this hormone in the CF gender dichotomy. |
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| Keywords: | Estrogen Cystic fibrosis CF gender gap |
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