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F508 CF mice by trimethylamine oxide
1 Children's Hospital Oakland Research Institute, Oakland 94609; and 2 Cardiovascular Research Institute, University of California, San Francisco, California 94143
This study was designed to test
the in vivo efficacy of the chemical chaperone trimethylamine oxide
(TMAO) in correcting the Cl
transport defect in a mouse
model of cystic fibrosis (CF). Rectal potential difference (RPD)
measurements were done in matched wild-type and 
F508 CF
mice. Mice were treated by subcutaneous injections of TMAO. Wild-type
mice demonstrated a forskolin-stimulated, Cl-dependent
hyperpolarization of 
6.4 ± 0.8 mV (n = 11),
which was significantly increased to 13.1 ± 1.4 mV after
treatment with TMAO. F508 CF mice showed no significant responses to
forskolin. Treatment with TMAO recovered a forskolin-activated RPD in
F508 CF mice (1.1 ± 0.2 mV; n = 17) but not
in CFTR null mice. The effects of TMAO were dose dependent, resulting
in a slope of 0.4 ± 0.1 mV · g1 · kg1 in F508 CF
mice. The forskolin-stimulated RPD in TMAO-treated F508 CF mice was
partially blocked by glibenclamide and further stimulated by apigenin.
The total response to forskolin plus apigenin was 2.5 ± 0.45 mV
(n = 6 mice), corresponding to 39% of the response evoked by forskolin only in wild-type mice.
apigenin; F508 cystic fibrosis transmembrane conductance
regulator; cystic fibrosis transmembrane conductance regulator
knockout; glibenclamide; rectum; epithelia
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