The cystic fibrosis transmembrane regulator (CFTR) in the kidney
An. acad. bras. ciênc
;
72(3): 399-406, Sept. 2000. ilus
Artículo
en Inglés
| 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:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Regulador de Conductancia de Transmembrana de Fibrosis Quística
/
Riñón
Límite:
Humanos
Idioma:
Inglés
Revista:
An. acad. bras. ciênc
Asunto de la revista:
Ciencia
Año:
2000
Tipo del documento:
Artículo
País de afiliación:
Brasil
Institución/País de afiliación:
Universidade Federal do Rio de Janeiro/BR
Similares
MEDLINE
...
LILACS
LIS