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. 2014 Sep 8;24(1):185–196. doi: 10.1093/hmg/ddu428

Figure 2.

Figure 2.

Cln1 mRNA expression and PPT1 enzyme activity is decreased in Cln1R151X mice. (A) Endogenous expression of Cln1 in the cerebellum (Cereb), cortex (Cortex), striatum/thalamus (StrThal), brainstem (BrStem) and liver from 5-month-old homozygous (white bars) and heterozygous (hashed bars) Cln1R151X mice is decreased compared with wild type (grey bars). Quantitative real-time PCR (qPCR) was used to measure endogenous expression of Cln1. The relative expression of Cln1 was normalized with four reference genes (B2m, Gapdh, Gusb, Hgprt). (B) Endogenous PPT1 enzyme activity in the cerebellum, cortex, striatum/thalamus, brainstem and liver from 5-month-old homozygous (white bars) and heterozygous (hashed bars) Cln1R151X mice is decreased compared with wild type (grey bars). All samples were compared with age- and sex-matched wild-type controls. Four technical replicates from each of three biological replicates were used for each genotype. All values are represented as mean ± SEM; *P < 0.05, **P < 0.01, ***P < 0.001.