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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. 1.

Fig. 1

Mutant Ubqln2 was predisposed to aggregation in vitro and in vivo. a Western blotting revealed that mutant Ubqln2 was enriched in Triton X-100 insoluble fraction. HEK293 cells were transiently transfected with plasmids expressing wildtype (WT) or mutant (MUT, P497H substitution) human Ubqln2 that was tagged with FLAG. Triton X-100 soluble and insoluble fractions were isolated as described in “Materials and methods” and displayed in b. b Shows the procedure to separate insoluble from soluble Ubqln2 in cultured cells (dissolved in 7 M urea) and transgenic rats (dissolved in 1 % SDS). c Western blotting revealed that the full-length and C-terminal fragments of Ubqln2 were enriched in Triton X-100 insoluble fraction. Frontal cortex was dissected from CaMKα2-tTA single-transgenic (tTA: the control) and CaMKα2-tTA/TRE-hUBQLN2-P497H double-transgenic rats (P497H) at the age of 80 days. Immunoreactivity was detected with an antibody that recognizes both human and rat Ubqln2