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1 Division of Nephrology, Université Catholique de Louvain Medical School, B-1200 Brussels, Belgium; and 2 Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
Cl
channels are
involved in a range of functions, including regulation of cell volume
and/or intracellular pH, acidification of intracellular vesicles, and
vectorial transport of NaCl across many epithelia. Numerous
Cl channels have been identified in the kidney, based on
single-channel properties such as conductance, anion selectivity,
gating, and response to inhibitors. The molecular counterpart of many
of these Cl channels is still not known. This review will
focus on gene-targeted mouse models disrupting two structural classes
of Cl channels that are relevant for the kidney: the CLC
family of voltage-gated Cl channels and the CFTR.
Disruption of several members of the CLC family in the mouse provided
useful models for various inherited diseases of the kidney, including
Dent's disease and diabetes insipidus. Mice with disrupted CFTR are
valuable models for cystic fibrosis (CF), the most common autosomal
recessive, lethal disease in Caucasians. Although CFTR is expressed in
various nephron segments, there is no overt renal phenotype in CF.
Analysis of CF mice has been useful to identify the role and potential
interactions of CFTR in the kidney. Furthermore, observations made in
CF mice are potentially relevant to all other models of
Cl channel knockouts because they emphasize the
importance of alternative Cl pathways in such models.
Bartter's syndrome; bicarbonate transport; chloride channel; cystic fibrosis transmembrane conductance regulator; Dent's disease; endocytosis; nephrogenic diabetes insipidus; vesicular acidification
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