Table 1.

Main animal models of progeria.

Model	Main Phenotypic Features	Main Limitations	Ref.
Zmpste24−/− mice	Bone fragility, reduced weight and growth, defective prelamin A processing, early death, muscular weakness, age-dependent cardiac electrical defects	No severe vascular alterations	[19,20,21]
Heterozygous LMNAp.G608G/+ mice	Ubiquitous progerin accumulation, vascular abnormalities	Lack rest of features of progeria phenotype	[22]
LmnaG609G/G609G mice	Ubiquitous progerin accumulation, shortened lifespan, reduced weight, main metabolic, bone, and cardiovascular alterations	Mice do not develop atherosclerosis	[23]
Apoe−/− LmnaG609G/G609G mice	Same phenotype as LmnaG609G/G609G but including the development of atherosclerosis	-	[25]
Apoe−/−LmnaLCS/LCS SM22αCre mice	Progerin expression restricted to VSMCs. Mice recapitulate vascular features of progeria	Lack of overt growth defects and other disease symptoms compared to the phenotype observed in LmnaG609G/G609G mice	[25]
Apoe−/−LmnaLCS/LCS LysMCre mice	Progerin expression restricted to macrophages	Lack of overt growth defects and other disease symptoms compared to the phenotype observed in LmnaG609G/G609G mice and in Apoe−/−LmnaLCS/LCS SM22αCre	[25]
Prog-Tg mice	Progerin expression restricted to endothelium. Reduced growth, weight, and lifespan. Mice recapitulate many cardiovascular alterations such as profibrotic response and cardiac functional impairment	Lack of VSMC loss	[26]
Knockin heterozygous LMNA c.1824C > T Yucatan minipig	Expression of progerin and normal lamin A/C, growth retardation, lipodystrophy, skin and bone alterations, cardiovascular alterations, cardiovascular disease, and mortality around puberty	Difficulty to establish an HGPS minipig colony through conventional breeding	[27]