Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.

"the specificity of both sets of antibodies for their native peptides was tested by elisa and by western blotting from cell lines expressing relevant dprs (data s1 and figures s1 and s2)."

"Conflict of interest All authors declare no conflict of interest."

"XL and DW are supported by the Project on Absorption of Intellects by Institutions of Higher Education for Academic Disciplinary Innovations (the ‘111 Project’) (no: B08006). We also acknowledge the support of the Manchester Brain Bank by Alzheimer's Research UK and Alzheimer's Society through their funding of Brains for Dementia Research (BDR) Programme, and Medical Research Council through service support costs. DMAM and SPB received funding from Medical Research Council and The Wellcome Trust (grant no. 089701/Z/09/Z), through their Neurodegeneration Research Initiative, which supported this study in part. The London Neurodegenerative Diseases Brain Bank receives funding from Medical Research Council."