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Phenotypes Associated with This Genotype
Genotype
MGI:3833377
Allelic
Composition
Fras1tm1Chpk/Fras1tm1Chpk
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * NMRI
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fras1tm1Chpk mutation (1 available); any Fras1 mutation (133 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Defective septation of right lung lobes in Fras1tm1Chpk/Fras1tm1Chpk embryos

respiratory system
• embryonic lung shows basement membrane distortions
• endothelial cells are locally detached from the basement membrane in the terminal sacs
• extravasation of erythrocytes into the embryonic respiratory lumen
• disarrangement of blood capillaries in the terminal air sacs of the lungs
• lobar septation defects are observed throughout embryogenesis and adulthood
• local disruptions of epithelial-endothelial contacts
• mutants exhibit completely or partially fused right lung lobes resulting from incomplete separation during development

cardiovascular system
• disarrangement of blood capillaries in the terminal air sacs of the lungs

cellular
• embryonic lung shows basement membrane distortions
• basement membranes between endothelial and epithelial type I cells in the lung appear wider and less dense compared with wild-type

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Fraser syndrome DOID:0090001 OMIM:PS219000
J:137010


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
09/10/2019
MGI 6.14
The Jackson Laboratory