Mouse Genome Informatics
ht
    Fbn1tm1Lper/Fbn1+
involves: 129/Sv * CD-1
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• at 3 months with hemothorax

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

cardiovascular system
• aortic media is thickened compared to in wild-type mice
• 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
• mice on a congenic 129/Sv background exhibit wide clinical variability

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
• mice on a congenic 129/Sv background exhibit wide clinical variability
• at 2 months

immune system
• chronic

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