FIGURE 1.
TDP-43 expression in heterozygous hTDP-43A315T mice. A, homozygous animals expressing human A315T TDP-43 were embryonic lethal at weaning age. At stage E7.5 no homozygous embryo was found by PCR genotyping. B, Western blots of various tissue lysates from a 6-month-old mutant mouse compared with control probed for total TDP-43 or human TDP-43 revealed a tissue-specific pattern as well as increased TDP-43 levels for all organs. C, Western blots of brain lysates from 1-, 6-, and 12-month-old mutants (+/−) and controls (wt) using antibodies against total TDP-43 and human TDP-43. D, quantification of mouse and human TDP-43 isoforms via quantitative real-time PCR. Down-regulation of endogenous mouse TDP-43 to about 20% as well as a 3–4-fold up-regulation of human TDP-43 in mutants compared with controls. Error bars, S.D.