Phenotypes Associated with This Genotype

Genotype
MGI:4839088

• Allelic Composition: Fbn1^tm1.2Lysa/Fbn1^tm1.2Lysa
• Genetic Background: involves: C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Perturbed microfibril structure in Fbn1^tm1.2Lysa/Fbn1^tm1.2Lysa mice

mortality/aging

postnatal lethality, complete penetrance ( J:165897 )

• mice die between P9 and P18 with most mice dying at P14

cardiovascular system

abnormal aorta elastic fiber morphology ( J:165897 )

• at 2 months, mice exhibit progressive fragmentation of the aortic elastic lamellae with age unlike wild-type mice

skeleton

abnormal tendon morphology ( J:165897 )

• at P8, mice exhibit fragmentation of the microfibril network unlike in wild-type mice

muscle

abnormal skeletal muscle fiber morphology ( J:165897 )

• at P8, mice exhibit fragmentation of the microfibril network unlike in wild-type mice

abnormal tendon morphology ( J:165897 )

• at P8, mice exhibit fragmentation of the microfibril network unlike in wild-type mice

integument

abnormal cutaneous microfibril morphology ( J:165897 )

• at P8, mice exhibit fragmentation of the microfibril network unlike in wild-type mice