Acinar Cell Apoptosis in Serpini2-Deficient Mice Models Pancreatic Insufficiency 英文参考文献.docVIP

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Acinar Cell Apoptosis in Serpini2-Deficient Mice Models Pancreatic Insufficiency 英文参考文献.doc

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Acinar Cell Apoptosis in Serpini2-Deficient Mice Models Pancreatic Insufficiency 英文参考文献

AcinarCellApoptosisinSerpini2-Deficient MiceModelsPancreaticInsufficiency Stacie K.Loftus1*,Jennifer L.Cannons1,Arturo Incao1,Evgenia Pak1,Amy Chen1,Patricia M.Zerfas2,Mark A.Bryant2, Leslie G.Biesecker1,Pamela L.Schwartzberg1,William J.Pavan1 1GeneticDiseaseResearchBranch,NationalHumanGenomeResearchInstitute,NationalInstitutesofHealth,Bethesda,Maryland,UnitedStatesofAmerica,2Divisionof VeterinaryResources,OfficeofResearchServices,NationalInstitutesofHealth,Bethesda,Maryland,UnitedStatesofAmerica Pancreatic insufficiency (PI) when left untreated results in a state of malnutrition due to an inability to absorb nutrients.Frequently,PIisdiagnosedaspartofalargerclinicalpresentationincysticfibrosisorShwachman–Diamond syndrome. In this study, a mouse model for isolated exocrine PI was identified in a mouse line generated by a transgene insertion. The trait is inherited in an autosomal recessive pattern, and homozygous animals are growth retarded, have abnormal immunity, and have reduced life span. Mice with the disease locus, named pequen?o (pq), exhibitprogressiveapoptosisofpancreaticacinarcellswithsevereexocrineacinarcelllossby8wkofage,whilethe islets and ductal tissue persist. The mutation in pq/pq mice results from a random transgene insertion. Molecular characterization of the transgene insertion site by fluorescent in situ hybridization and genomic deletion mapping identified an approximately 210-kb deletion on Chromosome 3, deleting two genes. One of these genes, Serpini2, encodesaproteinthatisamemberoftheserpinfamilyofproteaseinhibitors.ReintroductionofonlytheSerpini2gene bybacterialartificialchromosometransgeniccomplementationcorrectedtheacinarcelldefectaswellasbodyweight andimmunephenotypes,showingthatdeletionofSerpini2causesthepequen?ophenotype.Dietarysupplementation ofpancreaticenzymesalsocorrectedbodysize,bodyweight,andimmunodeficiency,andincreasedthelifespanof Serpini2-deficient mice, despite continued acinar cell loss. To our knowledge, this study