Hepatic iodothyronine deiodinase type 1 activity is decreased in two ΔF508 cystic fibrosis mouse models

Abstract

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 ΔF508 CF mouse models. Results: Hepatic D1 activity was significantly reduced by 31% to 48% in homozygous ΔF508 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.