TY - CHAP M1 - Book, Section TI - Cystic Fibrosis A1 - Sorscher, Eric J. A2 - Jameson, J. Larry A2 - Fauci, Anthony S. A2 - Kasper, Dennis L. A2 - Hauser, Stephen L. A2 - Longo, Dan L. A2 - Loscalzo, Joseph PY - 2018 T2 - Harrison's Principles of Internal Medicine, 20e AB - Cystic fibrosis (CF) is an autosomal recessive exocrinopathy affecting multiple epithelial tissues. The gene product responsible for CF (the cystic fibrosis transmembrane conductance regulator [CFTR]) serves as an anion channel in the apical (luminal) plasma membranes of epithelial cells and regulates volume and composition of exocrine secretion. An increasingly sophisticated understanding of CFTR molecular genetics and membrane protein biochemistry has facilitated CF drug discovery, with a number of new agents recently approved or advancing through the clinical testing phase. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1162605891 ER -