Figure 2
From: Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
Down regulation of mouse TDP-43 and production of lower molecular weight (LMW) TDP-43 species in TDP-43 M337V mice. Western blots (A) of brain lysates from NT, hemizygous (Hemi) and homozygous (Homo) TDP-43M337V mice probed for hTDP-43 or total TDP-43 levels were quantified by (B) densitometry to show that mutant hTDP-43 expression is approximately 90% higher in homozygous mice compared to hemizygous mice (Hemi = 1 ± 0.04; Homo = 1.9 ± 0.06), ** p < 0.001. Densitometric analysis (C) shows that total TDP-43 levels in each genotype group (NT = 1 ± 0.05; Hemi = 1.6 ± 0.03; Homo = 2.7 ± 0.08). ** p < 0.001. Data shown are means ± SEM of six different mice of each genotype. (D) Quantitative real time PCR (qPCR) using mTDP-43-specific primers demonstrate the mRNA levels of mTDP-43 in NT, hemizygous and homozygous mice brain (NT = 1 ± 0.07; Hemi = 0.8 ± 0.05; Homo = 0.7 ± 0.01). * p < 0.05, ** p < 0.01. (E) qPCR using hTDP-43 - specific primers shows the mRNA levels of hTDP-43 in hemizygous and homozygous mice brain (Hemi = 1 ± 0.09; Homo = 1.86 ± 0.14). ** p < 0.01. Data shown in (D) and (E) are means ± SEM of four different mice of each genotype. (F) Darker exposure of the Western blot shown in (A) revealed increased LMW species of TDP-43 in hemi- and homozygous TDP-43M337V mice. (G) Densitometric analysis of ~25 kDa and ~35 kDa TDP-43 species indicates that both species correlate with total TDP-43 expression (For ~25 kDa species, NT = 1 ± 0.14; Hemi = 1.4 ± 0.21; Homo = 3.4 ± 0.26. For ~35 kDa species, NT = 1 ± 0.32; Hemi = 1.6 ± 0.28; Homo = 2.4 ± 0.13). ** p < 0.01. Data shown are means ± SEM of six different mice of each genotype.