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. Author manuscript; available in PMC: 2016 Aug 1.
Published in final edited form as: Exp Gerontol. 2015 Jan 21;68:43–50. doi: 10.1016/j.exger.2015.01.045

Table 2.

Comparison of diverse mouse strains with accelerated aging due to genomic instability.

Ercc1-/- Ercc1-/Δ Bubr1H/H polγD257A/D257A
Human disease modeled XFE progeria syndrome XFE progeria syndrome Mosaic Variegated Aneuploidy None
Mutated gene Ercc1 Ercc1 Bubr1 Polg
Consequence of mutation Compromised in multiple DNA repair pathways Compromised in multiple DNA repair pathways Defective mitotic spindle assembly checkpoint Inability to proofread newly synthesized mtDNA
% Normal protein expression 0% 10% 10% 0%
Pathologies
Growth retardation* + + + -
Kyphosis + + + +
Sarcopenia + + + +
Skin changes& + - + +
Hematologic abnormalities# + - - +
SQ fat loss - - + +
Neurologic abnormalities˄ + + + -
Osteoporosis - + - +
Hearing loss - + - +
Hematopoietic organ atrophy and/or involution% - + - +
Impaired vision$ - + + -
Cardiovascular abnormalities - + + +
Other Liver failure Disc degeneration, renal dysfunction; infertility Impaired wound healing, facial dysmorphisms, cachexia, infertility Loss of intestinal crypts and intestinal dysplasia, infertility
Lifespan 3-4 weeks (McWhir and others 1993; Niedernhofer and others 2006; Weeda and others 1997) Median: 16 weeks; maximum: 29 weeks (Dolle and others 2011) Median: 6 months (Baker and others 2004) Median: 48 weeks; maximum: 61 weeks (Trifunovic and others 2004)
Median: 416 days; maximum: 460 days (Kujoth and others 2005)
Premature cell senescence? + + + -
*

Growth retardation includes runting and dwarfism

&

Skin changes include atrophy, reduced dermal thickness, hair graying, and alopecia

#

Hematologic abnormalities include leukopenia and thrombocytopenia

˄

Neurologic abnormalities include dystonia, tremors, ataxia, neurodegeneration, brain and/or spinal cord astrocytosis and microgliosis

%

Hematopoietic organs include spleen and thymus

$

Impaired vision includes cataracts

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