Document Detail


Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice.
MedLine Citation:
PMID:  20408952     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND/AIMS: Receptor-mediated endocytosis is a critical cellular mechanism for the uptake of lipoprotein cholesterol in the liver. Because Niemann-Pick C1 (NPC1) protein is a key component for the intracellular distribution of cholesterol originating from lipoprotein endocytosis, it may play an important role in controlling biliary cholesterol secretion and gallstone formation induced by a lithogenic diet. METHODS: We studied biliary cholesterol secretion, gallbladder lipid composition and gallstone formation in NPC1-deficient mice fed a low-fat lithogenic diet (1.5% cholesterol and 0.5% cholic acid) compared with control animals under the same diet. RESULTS: The lipid secretion response to the lithogenic diet was impaired in NPC1 (-/-) mice, leading to a decreased cholesterol output and an increased hepatic cholesterol concentration compared with the lithogenic diet-fed wild-type mice. A decreased cholesterol saturation index was found in the gallbladder bile of NPC1 (+/-) and (-/-) mice after lithogenic diet feeding. Consequently, mice with a partial or a total deficiency of NPC1 had a drastically lower frequency of gallbladder cholesterol crystals and a reduced prevalence of gallstones. CONCLUSION: Hepatic NPC1 expression is an important factor for regulating the biliary secretion of diet-derived cholesterol as well as for diet-induced cholesterol gallstone formation in mice.
Authors:
María Gabriela Morales; Ludwig Amigo; Elisa Balboa; Mariana Acuña; Juan Castro; Héctor Molina; Juan Francisco Miquel; Flavio Nervi; Attilio Rigotti; Silvana Zanlungo
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-04-08
Journal Detail:
Title:  Liver international : official journal of the International Association for the Study of the Liver     Volume:  30     ISSN:  1478-3231     ISO Abbreviation:  Liver Int.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-07-13     Completed Date:  2010-10-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101160857     Medline TA:  Liver Int     Country:  England    
Other Details:
Languages:  eng     Pagination:  887-97     Citation Subset:  IM    
Affiliation:
Departamento de Gastroenterología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
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MeSH Terms
Descriptor/Qualifier:
ATP-Binding Cassette Transporters / genetics
Animals
Bile / metabolism*
Biological Transport
Cholesterol 7-alpha-Hydroxylase / genetics
Cholesterol, Dietary / metabolism*
Cholic Acid
Disease Models, Animal
Gallstones / chemically induced,  genetics,  metabolism,  prevention & control*
Gene Expression Regulation
Hydroxymethylglutaryl CoA Reductases / genetics
Liver / metabolism*
Male
Mice
Mice, Inbred BALB C
Mice, Knockout
Proteins / genetics,  metabolism*
RNA, Messenger / metabolism
Receptors, LDL / genetics
Sterol Regulatory Element Binding Protein 2 / genetics
Vesicular Transport Proteins / genetics
Chemical
Reg. No./Substance:
0/ATP-Binding Cassette Transporters; 0/Cholesterol, Dietary; 0/Npc1 protein, mouse; 0/Npc2 protein, mouse; 0/Proteins; 0/RNA, Messenger; 0/Receptors, LDL; 0/Srebf2 protein, mouse; 0/Sterol Regulatory Element Binding Protein 2; 0/Vesicular Transport Proteins; 81-25-4/Cholic Acid; EC 1.1.1.-/Hydroxymethylglutaryl CoA Reductases; EC 1.14.13.17/Cholesterol 7-alpha-Hydroxylase; EC 1.14.13.17/cytochrome P450 7A1, mouse

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