Hepatic iodothyronine deiodinase type 1 activity is decreased in two DeltaF508 cystic fibrosis mouse models.
BACKGROUND
Abnormal thyroid status has been reported in cystic fibrosis (CF) patients, and this can possibly be correlated to neuromuscular symptoms. Iodothyronine deiodinase type 1 (D1) activity is an important determinant of thyroid status, and we chose to investigate D1 activity in CF liver.


METHODS
We have measured hepatic D1 activities in two DeltaF508 CF mouse models.


RESULTS
Hepatic D1 activity was significantly reduced by 31% to 48% in homozygous DeltaF508 mice compared with wild-type genotypes.


CONCLUSIONS
A decreased hepatic D1 activity could be the biochemical basis of some of the abnormal thyroid parameters observed in cystic fibrosis patients.
Klaren
Peter H M
PH
Department of Animal Physiology, Faculty of Science, University of Nijmegen, Toernooiveld 1, NL-6525 ED, Nijmegen, The Netherlands. pklaren@sci.kun.nl
Looijmans
Pieter H A
PH
D000818:Animals; D003550:Cystic Fibrosis / Q000378:metabolism*; D004195:Disease Models, Animal; D004789:Enzyme Activation; D007453:Iodide Peroxidase / Q000378:metabolism*; D008099:Liver / Q000201:enzymology*; D008297:Male; D051379:Mice; D018181:Mice, Inbred CFTR; D014284:Triiodothyronine / Q000097:blood
D014284:Triiodothyronine; C109812:iodothyronine deiodinase type I; D007453:Iodide Peroxidase