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Phenotypes Associated with This Genotype
Genotype
MGI:5441753
Allelic
Composition
Tg(LMNA*G608G)HClns/?
Genetic
Background
C57BL/6-Tg(LMNA*G608G)HClns/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(LMNA*G608G)HClns mutation (1 available)
phenotype observed in females
phenotype observed in males
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 ID Ref(s)
Hutchinson-Gilford Progeria Syndrome; HGPS 176670 J:107176


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
08/17/2016
MGI 6.05
The Jackson Laboratory