Fig 4. Decreased TPP1 activity in various Cln2^R207X/R207X mouse tissues.

Fluorogenic TPP1 enzyme activity assays were used to measure endogenous levels of TPP1 activity from five different tissues obtained from 1-month-old Cln2^+/+ (n = 3) and Cln2^R207X/R207X (n = 3) mice. Four technical replicates were performed using the three biological samples obtained from Cln2^+/+ and Cln2^R207X/R207X mice. Cln2^R207X/R207X TPP1 activity was normalized to Cln2^+/+ TPP1 activity. Columns and bars represent mean ± SEM. Statistical significance was determined using an unpaired t-test (** p < 0.01, ***p < 0.001, and **** p < 0.0001).