Mouse Genome Informatics
tg
    Tg(LMNA*G608G)HClns/?
C57BL/6-Tg(LMNA*G608G)HClns/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• fewer homozygotes born than expected

cardiovascular system
• progressive vascular smooth muscle cell loss starting at 5 momnths of age
• broken elastic fibers, thickened adventitia and medial layers, and proteoglycan and collagen accumulation in large arteries.
• calcification and severe vascular smooth muscle cell loss and extracellular matrix deposition of the large arteries after 12 months of age
• impaired blood pressure regulation with an abnormal response to the vasodilator, sodium nitroprusside

muscle
• progressive vascular smooth muscle cell loss starting at 5 momnths of age
• broken elastic fibers, thickened adventitia and medial layers, and proteoglycan and collagen accumulation in large arteries.
• calcification and severe vascular smooth muscle cell loss and extracellular matrix deposition of the large arteries after 12 months of age

reproductive system
• homozygous females are infertile

Mouse Models of Human Disease
OMIM IDRef(s)
Hutchinson-Gilford Progeria Syndrome; HGPS 176670 J:107176