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Phenotypes Associated with This Genotype
Genotype
MGI:6423604
Allelic
Composition
Lmnatm1Bliu/Lmna+
Tg(Tek-cre)1Ywa/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm1Bliu mutation (0 available); any Lmna mutation (76 available)
Tg(Tek-cre)1Ywa mutation (5 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• shorter median life span of 24 weeks
• mice treated with IS70, a recombinant AAV serotype 1 cassette with Sirt7 gene expression driven by a synthetic ICAM2 promoter show an expansion of median life span by 76%, from 25 to more than 44 weeks
• mice show accelerated aging in various tissues/organs
• mice treated with IS70 ameliorates aging features

growth/size/body
• mice exhibit weight loss
• mice treated with IS70 show a slight rescue in body weight loss

behavior/neurological
• running endurance is compromised

cardiovascular system
• severe fibrosis in the arteries
• intima-media thickening
• atherosclerosis is prominent, with aorta atheromatous plaque seen in all mice examined
• loss of CD31+ endothelial cells
• limb perfusion after ischemia is blunted; defect in blood flow recovery is due to an impaired ability to form new blood vessels in the ischemic region indicating defective neovascularization
• severe fibrosis in the heart
• heart/body weight ratio is increased, indicating dilated cardiomyopathy
• cardiac output is reduced in 7-8 month old mice
• left ventricular ejection fraction and fractional shortening are reduced in 7-8 month old mice
• heart rate is reduced in 7-8 month old mice
• acetylcholine-induced thoracic aorta relaxation is compromised

homeostasis/metabolism
• running endurance is compromised

muscle
• heart/body weight ratio is increased, indicating dilated cardiomyopathy
• left ventricular ejection fraction and fractional shortening are reduced in 7-8 month old mice
• acetylcholine-induced thoracic aorta relaxation is compromised

skeleton
• decrease in trabecular bone volume/tissue volume
• increase in trabecular separation

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
progeria DOID:3911 OMIM:176670
J:287256


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
09/22/2022
MGI 6.21
The Jackson Laboratory