| Ductal malformation and pancreatitis in mice caused by conditional Jag1 deletion. | |
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MedLine Citation:
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PMID: 19208348 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND & AIMS: Alagille syndrome is an autosomal dominant disorder caused by mutations in Notch signaling pathway genes, usually JAGGED1. Up to 40% of Alagille syndrome patients also display exocrine pancreatic insufficiency, the pathobiology of which is unknown. Additionally, no mouse model recapitulating this aspect of the disease has been reported. METHODS: We conditionally deleted both alleles of Jagged1 in the murine pancreas using Cre-loxP technology and analyzed histologic and morphologic features in postnatal and adult pancreas such as duct structure, acinar mass, and T-lymphocyte infiltration, as well as markers of pancreatic function, including fecal fat. RESULTS: Jagged1-deficient mice displayed malformed pancreatic ducts with resulting acinar cell death, fatty infiltration of the parenchyma, fibrosis, pancreatitis, and pancreatic insufficiency. CONCLUSIONS: Pancreatic ductal malformation and acinar cell loss may be responsible for pancreatic insufficiency in Jagged1-deficient mice and, by corollary, in Alagille syndrome patients. |
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Authors:
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Maria L Golson; Kathleen M Loomes; Rebecca Oakey; Klaus H Kaestner |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural Date: 2009-01-23 |
Journal Detail:
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Title: Gastroenterology Volume: 136 ISSN: 1528-0012 ISO Abbreviation: Gastroenterology Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-05-04 Completed Date: 2009-05-15 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0374630 Medline TA: Gastroenterology Country: United States |
Other Details:
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Languages: eng Pagination: 1761-71.e1 Citation Subset: AIM; IM |
Affiliation:
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Department of Genetics, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adipocytes
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pathology Alagille Syndrome / genetics Animals Calcium-Binding Proteins / genetics*, metabolism Gene Deletion* Glucose / metabolism Homeostasis Intercellular Signaling Peptides and Proteins / genetics*, metabolism Membrane Proteins / genetics*, metabolism Mice Mice, Knockout Mice, Mutant Strains Pancreas / metabolism, pathology Pancreatic Ducts / abnormalities*, pathology Pancreatitis / genetics*, metabolism, pathology |
| Grant Support | |
ID/Acronym/Agency:
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DK055342/DK/NIDDK NIH HHS; DK071/DK/NIDDK NIH HHS; P30DK19525/DK/NIDDK NIH HHS; P30DK50306/DK/NIDDK NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Calcium-Binding Proteins; 0/Intercellular Signaling Peptides and Proteins; 0/Membrane Proteins; 134324-36-0/Serrate proteins; 50-99-7/Glucose |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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