Figure 4.

Cln3^lacZ/lacZ mice have progressive behavioral deficits relative to age-matched Cln3^lacZ/+ control littermates. A, The rotarod assay was performed at the ages indicated and at 2, 6, and 10 months of age, Cln3^lacZ/lacZ mice exhibited significant impairment at all days tested (n = 11 for each genotype; p < 0.005). B, Nearly all Cln3^lacZ/lacZ mice display a clasping phenotype by 8 months of age (n = 11 for each genotype). C, Cln3^lacZ/lacZ mice show reduced nocturnal activity at 10 months of age compared with 2 months of age, and, at 10 months, the activity level of Cln3^lacZ/lacZ mice is significantly lower than Cln3^lacZ/+ controls (n = 11 for each genotype; p < 0.005 at 10 months). D, Cln3^lacZ/lacZ mice have a significant tremor phenotype relative to control mice (n = 11 for each genotype; p < 0.005 at all time points).