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Phenotypes Associated with This Genotype
Genotype
MGI:4457609
Allelic
Composition
Lmnatm5Lgf/Lmnatm5Lgf
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm5Lgf mutation (0 available); any Lmna mutation (63 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median survival of males and females is 38.5 weeks and 49.5 weeks, respectively
• all male mice succumb by 44 weeks, and all female mice succumb by 80 weeks of age

cellular
• nuclei of fibroblasts were misshapen, with blebs and folds

growth/size/body
• body weight of females are slightly but significantly lower than those of wild-type females

cardiovascular system
• during systole
• phenotype is more severe in males
• significant fibrosis in the papillary muscle of the left ventricle, as judged by a Gomori trichrome stain
• fibrosis is typically mild to moderate
• with an increased left ventricular (LV) chamber size during systole and a reduced LV ejection fraction, with a greater severity in males
• decrease in left ventricular ejection fraction
• decrease is more severe in males

muscle
• with an increased left ventricular (LV) chamber size during systole and a reduced LV ejection fraction, with a greater severity in males
• decrease in left ventricular ejection fraction
• decrease is more severe in males

skeleton
N
• do not develop osteolytic lesions or spontaneous rib fractures

Mouse Models of Human Disease
OMIM ID Ref(s)
NOT Hutchinson-Gilford Progeria Syndrome; HGPS 176670 J:160705


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
04/26/2016
MGI 6.03
The Jackson Laboratory