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. Author manuscript; available in PMC: 2016 Mar 3.
Published in final edited form as: Acta Neuropathol. 2014 Nov 12;129(3):417–428. doi: 10.1007/s00401-014-1367-y

Fig. 2.

Fig. 2

Mutant Ubqln2 aggressively formed inclusions in transgenic rats. a–p Immunostaining revealed that Ubqln2 progressively formed inclusions in the dentate gyrus (a–j, n–p) and cortex (k–m, q–s) in CaMKα2-tTA/TRE-hUBQLN2-P497H double-transgenic rats (P497H), but not in CaMKα2-tTA single-transgenic rats (tTA). Transgenic rats were deprived of Dox at birth to allow transgene expression and were examined at indicated ages (d days). Total Ubqln2 was detected with an antibody that recognized both human and rat Ubqln2 proteins (hrUB2), and rat Ubqln2 was detected with a rat-specific antibody (rUB2). q–s Confocal microscopy revealed that Ubqln2 inclusions were accumulated in the cytoplasm and nucleus of affected cells. Scale bars 100 μm (a–e) and 30 μm (f–p). t–u Immunoprecipitation (IP) followed by immunoblotting (IB) revealed that mutant human Ubqln2 (hUB2: P497H) bound to wildtype (WT) human (hUB2) and rat (rUB2) Ubqln2. Asterisk indicates IgG heavy chain. HEK293 cells were transfected with indicated plasmid and were analyzed 32 h after transfection