Phenotypes Associated with This Genotype

Genotype
MGI:5806144

• Allelic Composition: Lmna^tm12Lgf/Lmna^tm12Lgf
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

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

cardiovascular system

abnormal aorta tunica adventitia morphology ( J:234711 )

• adventitial fibrosis by 4 months of age

abnormal aorta smooth muscle morphology ( J:234711 )

• by 4 months of age, mice show a near-complete loss of smooth muscle cells in the media of the ascending aorta

• mice treated with an exon 11-33 antisense oligonucleotide for 3 months beginning at 1 month of age show improved aortic pathology with less adventitial fibrosis and more smooth muscle cells

muscle

abnormal aorta smooth muscle morphology ( J:234711 )

• by 4 months of age, mice show a near-complete loss of smooth muscle cells in the media of the ascending aorta

• mice treated with an exon 11-33 antisense oligonucleotide for 3 months beginning at 1 month of age show improved aortic pathology with less adventitial fibrosis and more smooth muscle cells

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
progeria		DOID:3911	OMIM:176670	J:234711