Fig. 1

C9orf72 model zebrafish display RNA foci in the nucleus. (a) Schematic representation of the transgene inserted into 2.2 zebrafish. A zebrafish ubiquitin promotor drives GFP-DPR expression. An hsp70 promotor then drives DsRed production as a read out of cellular stress. (b) In situ hybridisation of paraffin embedded sections of 10dpf 2.2–7 zebrafish using a Cy3-conjugated (red) GC probe (GGGGCC)X4 showed that RNA foci are present in the nuclei of muscle cells. Arrow heads denote RNA foci. Scale bar = 10 μm. (c) Quantification of RNA foci showed Immunofluorescence labelling of adult zebrafish muscle tissue showed that poly-GP DPR protein localises to the nucleus in 2.2–2 and 2.2–7 transgenic zebrafish