In the present study we investigated 11 cases of FTDP-17 associated with intronic mutations affecting exon 10, but did not find any changes in neuronal hnRNP E2 immunoreactivity or binding of hnRNP E2 to aggregated tau, implying that the functional disturbances leading to increased splicing of exon 10 in this form of FTLD are not mediated by changes in hnRNP E2. Here, PCBP2 is linked to semantic dementia.