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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Hhextm2Cwb
targeted mutation 2, Clifford W Bogue
MGI:3721426
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Hhextm1Cwb/Hhextm2Cwb
Tg(Alb1-cre)1Khk/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL MGI:3721920
cn2
Hhextm2Cwb/Hhextm2Cwb
Tg(Alb1-cre)1Khk/?
involves: 129S1/Sv * C57BL/6 * SJL MGI:3721919
cn3
Hhextm1Cwb/Hhextm2Cwb
Tg(Foxa3-cre)1Khk/?
involves: 129X1/SvJ * C57BL/6 * DBA * SJL MGI:3721922


Genotype
MGI:3721920
cn1
Allelic
Composition
Hhextm1Cwb/Hhextm2Cwb
Tg(Alb1-cre)1Khk/?
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hhextm1Cwb mutation (0 available); any Hhex mutation (16 available)
Hhextm2Cwb mutation (1 available); any Hhex mutation (16 available)
Tg(Alb1-cre)1Khk mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
liver/biliary system
• intrahepatic bile duct morphogenesis is abnormal and results in hepatic cysts
• at E18.5, the portal tracts are often surrounded by many irregular biliary duct-like structures/cysts
• mice exhibit a ductal plate malformation
• in the liver, the portal vein is surrounded by multiple cysts (likely of biliary cell origin) and irregular duct-like structures
• at E18.5, the portal tracts are often surrounded by many irregular biliary duct-like structures/cysts
• cysts present in the parenchymal tissue appear to form in the absence of a branch of the portal vein
• at 8 weeks, 24 of 26 mice have cysts and four of these mice have more than 50 visible cysts
• cysts are seen in all lobes of the liver, multioculated, almost all filled with a clear fluid, range in size from 0.5mm to 8mm, and worsen with age
• hepatic cysts are a result of abnormal morphogenesis of the intrahepatic bile duct

endocrine/exocrine glands
• intrahepatic bile duct morphogenesis is abnormal and results in hepatic cysts

growth/size/body
• at E18.5, the portal tracts are often surrounded by many irregular biliary duct-like structures/cysts
• cysts present in the parenchymal tissue appear to form in the absence of a branch of the portal vein
• at 8 weeks, 24 of 26 mice have cysts and four of these mice have more than 50 visible cysts
• cysts are seen in all lobes of the liver, multioculated, almost all filled with a clear fluid, range in size from 0.5mm to 8mm, and worsen with age
• hepatic cysts are a result of abnormal morphogenesis of the intrahepatic bile duct




Genotype
MGI:3721919
cn2
Allelic
Composition
Hhextm2Cwb/Hhextm2Cwb
Tg(Alb1-cre)1Khk/?
Genetic
Background
involves: 129S1/Sv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hhextm2Cwb mutation (1 available); any Hhex mutation (16 available)
Tg(Alb1-cre)1Khk mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
liver/biliary system
• by 43 weeks, mice have developed severe cystic disease

growth/size/body
• by 43 weeks, mice have developed severe cystic disease




Genotype
MGI:3721922
cn3
Allelic
Composition
Hhextm1Cwb/Hhextm2Cwb
Tg(Foxa3-cre)1Khk/?
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * DBA * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hhextm1Cwb mutation (0 available); any Hhex mutation (16 available)
Hhextm2Cwb mutation (1 available); any Hhex mutation (16 available)
Tg(Foxa3-cre)1Khk mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

liver/biliary system
• at E13.5, the extrahepatic bile duct (EHBD) is absent and normal EHBD epithelium is replaced by pseudostratified epithelium resembling the duodenum
• at E16.5, the duodenal-like mucosa extends from the duodenum to the liver hilum
• at E16.5, dilated hepatic ducts and biliary cysts lined by cuboidal epithelium are present at the liver hilum
• at E16.5, biliary cysts are present at the liver hilum
• at E10.5, gall bladder primordium could not be detected by Onecut1 staining
• at E16.5, the ventral liver periphery has lost its cohesive structure and bloods cells are trapped or pool in this region
• at E18.5, normal hepatic architecture is disrupted
• at E18.5, livers exhibit large dilated duct-like structures
• at E18.5, livers are extremely cystic with large dilated duct-like structures and many smaller cysts
• at E13.5, the liver is hypoplastic

endocrine/exocrine glands
• at E13.5, the extrahepatic bile duct (EHBD) is absent and normal EHBD epithelium is replaced by pseudostratified epithelium resembling the duodenum
• at E16.5, the duodenal-like mucosa extends from the duodenum to the liver hilum
• at E16.5, dilated hepatic ducts and biliary cysts lined by cuboidal epithelium are present at the liver hilum
• at E18.5, livers exhibit large dilated duct-like structures
• at E16.5, biliary cysts are present at the liver hilum
• at E10.5, gall bladder primordium could not be detected by Onecut1 staining

growth/size/body
• at E16.5, biliary cysts are present at the liver hilum
• at E18.5, livers are extremely cystic with large dilated duct-like structures and many smaller cysts





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last database update
04/23/2024
MGI 6.23
The Jackson Laboratory