A Novel Transgenic Rat Model for Spinocerebellar Ataxia Type 17 Recapitulates Neuropathological Changes and Supplies In Vivo Imaging Biomarkers

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Figure 2.
Figure 2.

Phenotype of SCA17 rats. A, Starting with 4 months of age, transgenic (tg) male rats (n = 20) had a significantly lower body weight than age-matched control animals (n = 22). In female rats, this body weight difference was observed at the age of 5 months [n = 4 for TBPQ64; n = 8 for wild-type (wt) rats]. *p < 0.05; ***p < 0.001. B, At the age of 8 months, transgenic male rats were easily distinguishable from their wild-type littermates, as they were leaner and much smaller in size. D, Clasping behavior is observed in TBPQ64 rats at the age of 8 months when suspended by the tail, whereas nontransgenic animals showed normal spreading behavior of the hindlimbs (C). E, F, Compared to control animals (E) heterozygous rats were poorly groomed (F) and had brownish, eschar resembling structures in the fur (arrows in the magnified inset).

  1. The Journal of Neuroscience vol. 33 no. 21 9068-9081
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