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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Jag1tm1Frad
targeted mutation 1, Freddy Radtke
MGI:3577993
Summary 11 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Jag1tm1Frad/Jag1tm1Frad
Tg(Cdh5-cre)7Mlia/0
B6.Cg-Jag1tm1Frad Tg(Cdh5-cre)7Mlia MGI:5447166
cn2
Jag1tm1Frad/Jag1tm1Frad
Tg(Tagln-cre)1Her/0
B6.Cg-Jag1tm1Frad Tg(Tagln-cre)1Her MGI:5447165
cn3
Jag1tm1Frad/Jag1tm1Frad
Tg(Cdh5-cre)7Mlia/0
Gt(ROSA)26Sortm1Sor/Gt(ROSA)26Sor+
involves: 129 * 129S4/SvJaeSor * FVB/N MGI:5447167
cn4
Jag1tm1Frad/Jag1tm1Frad
Tg(Mx1-cre)1Cgn/0
involves: 129 * C57BL/6 * CBA MGI:3578401
cn5
Jag1tm1Frad/Jag1tm1Frad
Notch1tm1Agt/Notch1tm1Agt
Tg(Mx1-cre)1Cgn/0
involves: 129 * C57BL/6 * CBA MGI:3578403
cn6
Dll1tm1Mjo/Dll1tm1Mjo
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523691
cn7
Dll1tm1Mjo/Dll1tm1Mjo
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523692
cn8
Dll1tm1Mjo/Dll1+
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523693
cn9
Dll1tm1Mjo/Dll1+
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523694
cn10
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523695
cn11
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
involves: 129/Sv * C57BL/6 * CD-1 MGI:5523696


Genotype
MGI:5447166
cn1
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Cdh5-cre)7Mlia/0
Genetic
Background
B6.Cg-Jag1tm1Frad Tg(Cdh5-cre)7Mlia
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Cdh5-cre)7Mlia mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• contrary to previous reports, viable mice are produced

cardiovascular system
• abnormal positioning of the aorta and pulmonary trunk
• abnormal positioning of the aorta and pulmonary trunk
• fragile and hemorrhaging in embryos and neonates
• thin and disorganized
• incomplete at E14.5
• large and hyperplastic (myxomatous)
• neonates exhibit bulges near the valve annulus
• cartilage nodules and calcification in the annulus of adults
• thick at P10 with lower nuclear density indicating changes in the extracellular matrix rather than increase in cell density
• increased outflow tract valve leaflet width in adults
• in right ventricle
• near the apex
• with severe regurgitation at the pulmonic valve

liver/biliary system
N
• mice exhibit normal bile duct formation

Mouse Models of Human Disease
OMIM ID Ref(s)
Alagille Syndrome 1; ALGS1 118450 J:189213




Genotype
MGI:5447165
cn2
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Tagln-cre)1Her/0
Genetic
Background
B6.Cg-Jag1tm1Frad Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most mice die within 2 days of birth with few surviving beyond 4 weeks
• most mice die within 2 days of birth with few surviving beyond 4 weeks

liver/biliary system
• multifoci
• absent or incompletely formed bile ducts near the portal veins
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• epithelial cells fail to aggregate
• accumulation of bile in liver parenchyma
• at birth

homeostasis/metabolism
• total and conjugated
• increased circulating gamma-glutamyl transferase

growth/size/body
• at birth and in mice that survive beyond 4 weeks, worsening over time

behavior/neurological
• in mice that survive beyond 4 weeks, worsening over time

cardiovascular system
• multifoci

integument
• in mice that survive beyond 4 weeks, worsening over time

endocrine/exocrine glands
• absent or incompletely formed bile ducts near the portal veins
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• epithelial cells fail to aggregate

Mouse Models of Human Disease
OMIM ID Ref(s)
Alagille Syndrome 1; ALGS1 118450 J:166890




Genotype
MGI:5447167
cn3
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Cdh5-cre)7Mlia/0
Gt(ROSA)26Sortm1Sor/Gt(ROSA)26Sor+
Genetic
Background
involves: 129 * 129S4/SvJaeSor * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1Sor mutation (7 available); any Gt(ROSA)26Sor mutation (338 available)
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Cdh5-cre)7Mlia mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at E10.5, atrioventricular cushions exhibit rounded endocardial-derived cells that accumulate at the cushion interface and less dense, hypoplastic areas unlike in control mice




Genotype
MGI:3578401
cn4
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Mx1-cre)1Cgn/0
Genetic
Background
involves: 129 * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Mx1-cre)1Cgn mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
N
• homozygous mutant mice exhibit normal hematopoiesis, including normal hematopoietic stem cell self-renewal and differentiation




Genotype
MGI:3578403
cn5
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Notch1tm1Agt/Notch1tm1Agt
Tg(Mx1-cre)1Cgn/0
Genetic
Background
involves: 129 * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Notch1tm1Agt mutation (0 available); any Notch1 mutation (61 available)
Tg(Mx1-cre)1Cgn mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
N
• homozygous mutant mice exhibit normal hematopoiesis, including normal hematopoietic stem cell self-renewal and differentiation




Genotype
MGI:5523691
cn6
Allelic
Composition
Dll1tm1Mjo/Dll1tm1Mjo
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dll1tm1Mjo mutation (1 available); any Dll1 mutation (22 available)
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• nephron number is severely compromised, but some podocytes form




Genotype
MGI:5523692
cn7
Allelic
Composition
Dll1tm1Mjo/Dll1tm1Mjo
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dll1tm1Mjo mutation (1 available); any Dll1 mutation (22 available)
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• almost all nephrons are absent




Genotype
MGI:5523693
cn8
Allelic
Composition
Dll1tm1Mjo/Dll1+
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dll1tm1Mjo mutation (1 available); any Dll1 mutation (22 available)
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• podocytes are almost entirely absent; one allele of Dll1 cannot support podocyte production




Genotype
MGI:5523694
cn9
Allelic
Composition
Dll1tm1Mjo/Dll1+
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dll1tm1Mjo mutation (1 available); any Dll1 mutation (22 available)
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
N
• nephron development appears essentially normal




Genotype
MGI:5523695
cn10
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• nephron number is highly reduced




Genotype
MGI:5523696
cn11
Allelic
Composition
Jag1tm1Frad/Jag1+
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: 129/Sv * C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (35 available)
Tg(Six2-EGFP/cre)1Amc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
N
• nephron development appears essentially normal





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last database update
11/22/2016
MGI 6.06
The Jackson Laboratory