Article
Pathophysiological basis of liver disease in cystic fibrosis employing a DeltaF508 mouse model.
American journal of physiology. Gastrointestinal and liver physiology - 1 Jun 2008
Freudenberg Folke, Broderick Annemarie L, Yu Bian B, Leonard Monika R, Glickman Jonathan N, Carey Martin C
Abstract excerpt
The molecular pathogenesis of cystic fibrosis (CF) liver disease is unknown. This study investigates its earliest pathophysiological manifestations employing a mouse model carrying DeltaF508, the commonest human CF mutation. We hypothesized that, if increased bile salt spillage into the colon occurs as in the human disease, then this should lead to a hydrophobic bile salt profile and to "hyperbilirubinbilia"...
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