TY - JOUR AU - Davidson, Yvonne S. AU - Flood, Louis AU - Robinson, Andrew C. AU - Nihei, Yoshihiro AU - Mori, Kohji AU - Rollinson, Sara AU - Richardson, Anna AU - Benson, Bridget C. AU - Jones, Matthew AU - Snowden, Julie S. AU - Pickering-Brown, Stuart AU - Haass, Christian AU - Lashley, Tammaryn AU - Mann, David M. A. PY - 2017 DA - 2017/04/21 TI - Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene JO - Acta Neuropathologica Communications SP - 31 VL - 5 IS - 1 AB - Frontotemporal Lobar Degeneration (FTLD) encompasses certain related neurodegenerative disorders which alter behaviour, personality and language. Heterogeneous ribonuclear proteins (hnRNPs) maintain RNA metabolism and changes in their function may underpin the pathogenesis of FTLD. Immunostaining for hnRNP A1, A2/B1 and A3 was performed on sections of temporal cortex with hippocampus from 61 patients with FTLD, stratified by pathological hallmarks into FTLD-tau and FTLD-TDP type A, B and C subtypes, and by genetics into patients with C9orf72 expansions, MAPT or GRN mutations, or those without known mutation. Four patients with Motor Neurone Disease (MND) with C9orf72 expansions and 10 healthy controls were also studied. Semi-quantitative analysis assessed hnRNP staining intensity in dentate gyrus (DG) and CA4 region of hippocampus, and temporal cortex (Tcx) in the different pathological and genetic groups. SN - 2051-5960 UR - https://doi.org/10.1186/s40478-017-0437-5 DO - 10.1186/s40478-017-0437-5 ID - Davidson2017 ER -