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Phenotypes Associated with This Genotype
Genotype
MGI:3055916
Allelic
Composition
Onecut1tm1Flem/Onecut1tm1Flem
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * Swiss
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Mouse lines carrying:
Onecut1tm1Flem mutation (0 available); any Onecut1 mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
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 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
• at birth

growth/size/body
• 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 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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
01/28/2026
MGI 6.24
The Jackson Laboratory