The Cystic Fibrosis Transmembrane Regulator (CFTR) in the kidney

Anais da Academia Brasileira de Ciências

Marcelo M. Morales²  Doris Falkenstein¹  AnÍbal Gil Lopes² 

[1] ,Universidade Federal do Rio de Janeiro Instituto de Biofísica Carlos Chagas Filho Rio de Janeiro RJ ,Brazil

关键词: CFTR;    kidney;    nephron;    chloride channel;   

DOI  :  10.1590/S0001-37652000000300013

来源: SciELO

【 摘 要 】

The cystic fibrosis transmembrane regulator (CFTR) is a Cl- channel. Mutations of this transporter lead to a defect of chloride secretion by epithelial cells causing the Cystic Fibrosis disease (CF). In spite of the high expression of CFTR in the kidney, patients with CF do not show major renal dysfunction, but it is known that 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 the distal tubule, and the principal cells of the cortical (CCD) and medullary (IMCD) collecting ducts. Several studies have demonstrated that CFTR does not only transport Cl- but also secretes ATP and, thus, controls other conductances such as Na+ (ENaC) and K+ (ROMK2) channels, especially in CCD. In the polycystic kidney 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 volume regulators, such as hormones, on its function and expression.

【 授权许可】

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