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Severe-SMA mice harbouring two SMN2 copies, or with an extra SMNΔ7 cDNA transgene (SMA Δ7 mouse model), develop early and rapidly progressive pathology, dying within 1 2 weeks postnatally (Hsieh-Li et al, 2000; Le et al, 2005; Monani et al, 2000; Riessland et al, 2010).

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This sirtuin gained prominence when its knockout mouse model developed severe premature aging phenotypes with mortality resulting within a month (Mostoslavsky et al., 2006).

The 3xTg-AD mouse model develops both Aβ plaques and neurofibrillary tangles, the neuro-pathological hallmarks of AD, by 6 months of age, but no research has investigated the age-related changes in WM in these mice.

3xTg-AD mouse model develops two age-related neuropathological features associated with AD, amyloid plaques and neurofibrillary tangle formation, as well as age-related behavioral deficits that correlate with the neuropathology.

The FFD mouse model develops features of human NAFLD.

This mouse model developed ALS-like phenotype and pathology.

The Peg3 knock-out mouse model develops increased adiposity despite lower food intake.

This mouse model develops lung adenocarcinomas with a high incidence of metastases and gender differences in cancer-related death.

Finally, of considerable interest and wide attention is the triple tg mouse model developed by LaFerla et al.

For this purpose, we took advantage of a mouse model developed by endonasal administration of LAM/TSC cells in nude mice [ 28].

However, no mouse model developed thus far recapitulates all of these characteristics or exactly mimics what is seen in human cGvHD.

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