The cystic fibrosis transmembrane regulator (CFTR) in the kidney
An. acad. bras. ciênc
;
72(3): 399-406, Sept. 2000. ilus
Artigo
em Inglês
| LILACS
| ID: lil-269391
RESUMO
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.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Regulador de Condutância Transmembrana em Fibrose Cística
/
Rim
Limite:
Humanos
Idioma:
Inglês
Revista:
An. acad. bras. ciênc
Assunto da revista:
Ciência
Ano de publicação:
2000
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
Universidade Federal do Rio de Janeiro/BR
Similares
MEDLINE
...
LILACS
LIS