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Phenotypes Associated with This Genotype
Genotype
MGI:4880670
Allelic
Composition
Fbn1tm1Lper/Fbn1+
Genetic
Background
involves: 129/Sv * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fbn1tm1Lper mutation (0 available); any Fbn1 mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• at 3 months with hemothorax (J:167276)
• at 3 months with hemothorax (J:167276)

respiratory system
• chronic (J:167276)
• chronic (J:167276)
• at 3 months, pulmonary alterations in mice on a 129/Sv congenic background are stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)
• at 3 months, pulmonary alterations in mice on a 129/Sv congenic background are stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)
• mice exhibit enlarged peripheral air space (respiratory bronchioles and alveoli) compared with wild-type mice (J:167276)
• mice exhibit enlarged peripheral air space (respiratory bronchioles and alveoli) compared with wild-type mice (J:167276)
• mice exhibit enlarged peripheral air space (respiratory bronchioles and alveoli) compared with wild-type mice (J:167276)
• mice exhibit enlarged peripheral air space (respiratory bronchioles and alveoli) compared with wild-type mice (J:167276)

cardiovascular system
• aortic media is thickened compared to in wild-type mice (J:167276)
• Background Sensitivity: at 3 months, enlarged media in mice on a 129/Sv congenic background is stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)
• aortic media is thickened compared to in wild-type mice (J:167276)
• Background Sensitivity: at 3 months, enlarged media in mice on a 129/Sv congenic background is stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)

skeleton
• Background Sensitivity: skeletal manifestations (measured by the ratio between the linear distance and the length from the first cervical vertebrae to the last thoracic vertebrae) in mice on a 129/Sv congenic background are stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)
• Background Sensitivity: skeletal manifestations (measured by the ratio between the linear distance and the length from the first cervical vertebrae to the last thoracic vertebrae) in mice on a 129/Sv congenic background are stronger than in mice on a C57BL/6 congenic background (J:167276)
• mice on a congenic 129/Sv background exhibit wide clinical variability (J:167276)
• at 2 months (J:167276)
• at 2 months (J:167276)

immune system
• chronic (J:167276)
• chronic (J:167276)

Mouse Models of Human Disease
OMIM ID Ref(s)
Marfan Syndrome; MFS 154700 J:167276


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory