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Bibliographic Details
Main Author: Marcelo M. Morales
Format: Artículo científico
Language:en
Published: Academia Brasileira de Ciências 2000
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Online Access:https://www.redalyc.org/articulo.oa?id=32772313
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  • The Cystic Fibrosis Transmembrane Regulator (CFTR) in the Kidney Marcelo M. Morales Doris Falkenstein Aníbal Lopes Gil Multidisciplinaria (Ciencias Naturales y Exactas) CFTR kidney nephron chloride channel The cystic fibrosis transmembrane regulator (CFTR) is a Cl− channel. Mutations of this transporter lead toa defect of chloride secretion by epithelial cells causing the Cystic Fibrosis disease (CF). In spite of the highexpression of CFTR in the kidney, patients with CF do not show major renal dysfunction, but it is knownthat both the urinary excretion of drugs and the renal capacity to concentrate and dilute urine is deficient.CFTR mRNA is expressed in all nephron segments and its protein is involved with chloride secretion in thedistal tubule, and the principal cells of the cortical (CCD) and medullary (IMCD) collecting ducts. Severalstudies have demonstrated that CFTR does not only transport Cl− but also secretes ATP and, thus, controlsother conductances such as Na+ (ENaC) and K+ (ROMK2) channels, especially in CCD. In the polycystickidney the secretion of chloride through CFTR contributes to the cyst enlargement.This review is focused on the role of CFTR in the kidney and the implications of extracellular volumeregulators, such as hormones, on its function and expression. 2000 artículo científico 0001-3765 https://www.redalyc.org/articulo.oa?id=32772313 en http://www.redalyc.org/revista.oa?id=327 Anais da Academia Brasileira de Ciências application/pdf Academia Brasileira de Ciências Anais da Academia Brasileira de Ciências (Brasil) Num.3 Vol.72