Mouse Genome Informatics
cn1
    Foxa1tm1Khk/Foxa1tm1Khk
Foxa2tm1Khk/Foxa2+
Tg(Pdx1-cre)6Cvw/0

involves: 129 * C57BL/6 * CBA * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
endocrine/exocrine glands
N
• unlike in mice lacking both alleles of Fox2a, pancreatic development is normal (J:143476)

homeostasis/metabolism
N
• mice are euglycemic (J:143476)


Mouse Genome Informatics
cn2
    Foxa1tm1Khk/Foxa1+
Foxa2tm1Khk/Foxa2tm1Khk
Tg(Pdx1-cre)6Cvw/0

involves: 129 * C57BL/6 * CBA * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• mice die prior to P5

endocrine/exocrine glands
• the pancreas occupies 31% relative area compared to in wild-type mice

digestive/alimentary system


Mouse Genome Informatics
cn3
    Foxa1tm1Khk/Foxa1tm1Khk
Foxa2tm1Khk/Foxa2tm1Khk
Tg(Pdx1-cre)6Cvw/0

involves: 129 * C57BL/6 * CBA * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• mice die within 2 days of birth

endocrine/exocrine glands
• the spaces between the remaining ductal epithelia are filled with stromal tissue, including fibroblasts and smooth musce cells
• very few beta cells are present between E17.5 and P1
• at E13.5, pancreatic precursor cells fail to expand resulting in fewer than normal glucagon+ and amylase+ cells
• pancreatic development is arrested at the early pancreatic cord stage
• the pancreas occupies 19% relative area compared to in wild-type mice
• the numbers of endocrine cells, exocrine cells, and total epithelial tissue is severely decreased compared to in wild-type mice with remaining cells exhibiting an almost exclusively ductal phenotype

homeostasis/metabolism

digestive/alimentary system


Mouse Genome Informatics
cn4
    Sox9tm1Gsr/Sox9+
Tg(Pdx1-cre)6Cvw/0

involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * CBA * FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
endocrine/exocrine glands
N
• despite islet cell hypoplasia, pancreas size, weight, morphology and pancreatic exocrine differentiation are normal at E18.5 (J:141017)
• at E18.5, the numbers of polypeptide producing cells are reduced compared to wild-type mice
• at E18.5, alpha cell mass is reduced 55% compared to in wild-type mice
• at E18.5, beta cell mass is reduced 57% compared to in wild-type mice
• however, beta cell differentiation is normal
• at E18.5, delta cell numbers are decreased compared to in wild-type mice
• mice exhibit islet hyperplasia
• the number of pancreatic endocrine progenitor cells is 2-fold less than in wild-type mice due to decreased cell proliferation of endocrine progenitor cells
• however, exocrine progenitor cells are normal in number and proliferation
• pancreatic glucagons is reduced 34.9% compared to in wild-type
• pancreatic insulin production is decreased 44% compared to in wild-type mice

digestive/alimentary system
N
• despite islet cell hypoplasia, pancreas size, weight, morphology and pancreatic exocrine differentiation are normal at E18.5 (J:141017)

homeostasis/metabolism
• pancreatic glucagons is reduced 34.9% compared to in wild-type
• pancreatic insulin production is decreased 44% compared to in wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Campomelic Dysplasia 114290 J:141017


Mouse Genome Informatics
cn5
    Gt(ROSA)26Sortm1(ptxA)Cgh/Gt(ROSA)26Sor+
Tg(Pdx1-cre)6Cvw/0

involves: 129P2/OlaHsd * C57BL/6 * CBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
homeostasis/metabolism
• glucose clearance is increased compared to in wild-type mice


Mouse Genome Informatics
cn6
    Pax6tm2Pgr/Pax6tm2Pgr
Tg(Pdx1-cre)6Cvw/0

involves: FVB * NMRI
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• most mutant pups die after birth

endocrine/exocrine glands
• very few alpha cells are seen
• the number of insulin-expressing cells is decreased