Document Detail


Pathophysiological preconditions promoting mixed "black" pigment plus cholesterol gallstones in a DeltaF508 mouse model of cystic fibrosis.
MedLine Citation:
PMID:  20430874     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Gallstones are frequent in patients with cystic fibrosis (CF). These stones are generally "black" pigment (i.e., Ca bilirubinate) with an appreciable cholesterol admixture. The pathophysiology and molecular mechanisms for this "mixed" gallstone in CF are unknown. Here we investigate in a CF mouse model with no overt liver or gallbladder disease whether pathophysiological changes in the physical chemistry of gallbladder bile might predict the occurrence of "mixed" cholelithiasis. Employing a DeltaF508 mouse model with documented increased fecal bile acid loss and induced enterohepatic cycling of bilirubin (Am J Physiol Gastrointest Liver Physiol 294: G1411-G1420, 2008), we assessed gallbladder bile chemistry, morphology, and microscopy in CF and wild-type mice, with focus on the concentrations and compositions of the common biliary lipids, bilirubins, Ca(2+), and pH. Our results demonstrate that gallbladder bile of CF mice contains significantly higher levels of all bilirubin conjugates and unconjugated bilirubin with lower gallbladder bile pH values. Significant elevations in Ca bilirubinate ion products in bile of CF mice increase the likelihood of supersaturating bile and forming black pigment gallstones. The risk of potential pigment cholelithogenesis is coupled with higher cholesterol saturations and bile salt hydrophobicity indexes, consistent with a proclivity to cholesterol phase separation during pigment gallstone formation. This is an initial step toward unraveling the molecular basis of CF gallstone disease and constitutes a framework for investigating animal models of CF with more severe biliary disease, as well as the human disease.
Authors:
Folke Freudenberg; Monika R Leonard; Shou-An Liu; Jonathan N Glickman; Martin C Carey
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2010-04-29
Journal Detail:
Title:  American journal of physiology. Gastrointestinal and liver physiology     Volume:  299     ISSN:  1522-1547     ISO Abbreviation:  Am. J. Physiol. Gastrointest. Liver Physiol.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-06-16     Completed Date:  2010-07-12     Revised Date:  2012-05-02    
Medline Journal Info:
Nlm Unique ID:  100901227     Medline TA:  Am J Physiol Gastrointest Liver Physiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  G205-14     Citation Subset:  IM    
Affiliation:
Department of Medicine, Harvard Medical School and Harvard Digestive Diseases Center; Department of Medicine, Gastroenterology Division, Brigham and Women's Hospital, 75 Francis St., Boston, MA 02115, USA.
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MeSH Terms
Descriptor/Qualifier:
Animals
Bile / metabolism*
Bilirubin / metabolism*
Cholelithiasis / etiology*,  genetics,  metabolism,  pathology,  physiopathology
Cholesterol / metabolism*
Cystic Fibrosis / complications*,  genetics,  metabolism,  pathology,  physiopathology
Disease Models, Animal
Enterohepatic Circulation
Feces / chemistry
Female
Gallbladder / metabolism,  pathology,  physiopathology*
Gallstones / etiology*,  genetics,  metabolism,  pathology,  physiopathology
Hydrogen-Ion Concentration
Hydrophobic and Hydrophilic Interactions
Male
Mice
Mice, Inbred CFTR
Mucins / metabolism
Risk Factors
Grant Support
ID/Acronym/Agency:
DK-036588/DK/NIDDK NIH HHS; DK-073687/DK/NIDDK NIH HHS; R01 DK073687/DK/NIDDK NIH HHS; R01 DK073687-03/DK/NIDDK NIH HHS; R37 DK036588/DK/NIDDK NIH HHS; R37 DK036588-24/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/Mucins; 57-88-5/Cholesterol; 635-65-4/Bilirubin
Comments/Corrections

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