Fig 1: FG NUPs colocalize with TDP-43 proteinopathy in ALS/FTLD.NUP98, NUP54 or NUP62 (green) and total TDP-43, p62 or phosphoTDP-43 (pTDP-43, red) immunohistochemistry in postmortem tissue from various clinical diagnoses. a Cytoplasmic NUP98 is present in cells with cytoplasmic total TDP-43 (arrow) of C9-ALS/FTD postmortem hippocampal tissue. b Cytoplasmic NUP98 exhibits occasional colocalization (arrow) with p62+ inclusions in C9-ALS/FTD postmortem hippocampal tissue. However, cytoplasmic NUP98 was also found absent from p62+ inclusions (asterisks) in C9-ALS/FTD hippocampal tissue. c Phosphorylated TDP-43 was absent of cytoplasmic NUP54 in C9-ALS/FTD postmortem hippocampal tissue. d Postmortem sALS patient spinal cord tissue shows pTDP-43 inclusions that are both positive (arrow) and negative (asterisks) for NUP54. e, f Intact nuclear NUP62 signal surrounds DAPI+ nuclei (asterisks) but becomes more diffuse in cells exhibiting cytoplasmic pTDP-43 (arrow). Colocalization of NUP62 and pTDP-43 accumulations were observed in the spinal cord (E) and mesial temporal cortex (F) of sporadic ALS patient tissue. g Cytoplasmic FUS accumulations (red, asterisks) in the hippocampus from two FTLD patients do not show NUP62 localization. Scale bar: 10 µm.
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