Figure 2.

Storage Material and TPP1 Activity

Significance of changes in SCMAS and TPP1 activity relative to vehicle-treated LINCL mice was calculated by fitting data to a linear model, as described in the Materials and Methods. (A) Detection of SCMAS in isolated storage material by immunoblotting in animals treated with a weekly administration of 2.3 mg rhTPP1 and controls. Samples from 16-week-old wild-type and LINCL animals are shown as reference standards. Analysis of older wild-type animals indicated that there were no detectable age-related changes in SCMAS levels (data not shown). Relative SCMAS levels obtained from multiple loadings (4 and 8 μg protein equivalents of original homogenate) were normalized to average signal obtained from 17-week-old wild-type animals; error bars show SEM (n = 3 per treatment group). Significance of change compared to vehicle-treated LINCL mice, *p < 0.05/3 (i.e., 0.05 after Bonferroni correction for three individual comparisons). (B) TPP1 activities measured in brain, 24 hours after administration of the final dose. Data are expressed relative to average TPP1 activity in 17-week-old wild-type animals. Significance of change compared to vehicle treated animals, *p < 0.05/7 (i.e., 0.05 after Bonferroni correction for seven individual comparisons).