Fig. 5.
Accumulated UBQLN2 leads to the abnormal accumulation of autophagy-related proteins. a Double staining f p62 and UBQLN2 reveals that p62 progressively accumulates in the ventral horns of the spinal cord in ChATtTA/UBQLN2P497H (P497H) but not in ChATtTA single transgenic (ChATtTA) rats. Accumulated p62 colocalizes with UBQLN2 inclusions. The arrows point to the protein inclusions. m: months. b Double staining of LC3 and UBQLN2 reveals weaker staining of LC3 in P497H rats than in ChATtTA rats. c Western blots of spinal cord lysates at 1 month, 6 months, and 12 months old probed with the indicated antibodies. d Graphs showing the quantification of human UBQLN2, P62, LAMP2a, LC3-I, LC3-II, LC3-II/LC3-I, GFAP, TDP-43 and Iba1, all of which are shown in (c). The data are reported as the mean ± standard deviation (n = 3, male rats were used). e Immunofluorescence staining shows that LAMP2a is accumulated in P497H but not ChATtTA rats at 6-month old. Scale bars: 50 μm