mortality/aging
|
• 75% die between day 1 and day 10
• remaining 25% reach adulthood
|
liver/biliary system
|
• enlarged structures form connecting liver to duodenum rather than individualized hepatic ducts
• duct-like structures formed around E14.5 becoming large cysts by E16.5
• almost completely regressed by age 10 days
|
biliary cyst
(
J:100352
)
|
• biliary cysts are seen in the vicinity of the portal vein in embryos
|
|
• gall bladder missing as early as E10.5
|
|
• hepatoblast differentiation is perturbed such that they differentiate into hybrid cells that display characteristics of both hepatocytes and biliary cells
|
growth/size/body
biliary cyst
(
J:100352
)
|
• biliary cysts are seen in the vicinity of the portal vein in embryos
|
|
• reduced growth rate after birth
|
|
• growth retarded at birth
|
endocrine/exocrine glands
|
• enlarged structures form connecting liver to duodenum rather than individualized hepatic ducts
• duct-like structures formed around E14.5 becoming large cysts by E16.5
• almost completely regressed by age 10 days
|
biliary cyst
(
J:100352
)
|
• biliary cysts are seen in the vicinity of the portal vein in embryos
|
|
• gall bladder missing as early as E10.5
|
|
• number of Neurog3 expressing cells is reduced relative to controls at E14.5; pancreas sections almost completely have 4 Neurog3-expressing cells/section compared to 56 cells/section in wild-type
• very few insulin or glucagon-expressing cells are detected
|
|
• Islets do not become organized until 2-3 weeks of age
|
|
• number of glucagon expressing cells is reduced 85% at E12.5 and E15.5
• number of glucagon cell clusters is normal at the same time
• marked reduction in the number of alpha cells at 4 days of age
|
|
• glucagon-expressing cells are almost absent
|
|
• marked reduction in numbers of beta cells at 4 days of age
|
|
• insulin-expressing cells are almost absent
|
|
• marked reduction in the number of delta cells at 4 days of age
|
|
• abnormal architecture, alpha cells scattered rather than forming a peripheral mantle
|
|
• at about E10.5, ventral pancreatic bud is missing and dorsal pancreas is smaller relative to wild-type controls
(J:62595)
• at E13.5, pancreatic buds have not fused, dorsal pancreas is smaller than in controls, and the ventral pancreas is only a minute bud in the duodenal loop
(J:62595)
• endocrine differentiation initiates normally at E9.5 and progresses comparably to wild-type at E10.5, but becomes defective by E12.5
(J:62595)
• at E13.5, dorsal pancreas is smaller than in Onecut1-deficient embryos; no ventral bud is observed, instead consisting of two small cell clusters on the duodenum
(J:121610)
|
|
• endocrine differentiation is more affected than in Onecut1-null embryos; pancreas sections almost completely lack Neurog3-expressing cells (~0.5 cells/section)
• endocrine differentiation initiates normally at E9.5 and progresses comparably to wild-type at E10.5, but becomes defective by E12.5
|
|
• mice show pancreas hypoplasia during development
|
homeostasis/metabolism
|
• blood glucose low in fasted 4 day old mice
|
|
• at 10 weeks, elevated fasting glycemia in a glucose tolerance test
|
|
• glucagons levels reduced 20% in 4 day old fasted mice
|
|
• no insulin at E15.5
• insulin levels undetectable in 4 day old fasted mice
|
|
• at 10 weeks of age
|


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