Fig. 3.

TDP-43/UBQLN2 inclusions at 8 months of age. a Immunofluorescence of lumbar ventral horn at 5 and 8 months of age using TDP-43 antibody at × 20 and × 40 magnification (scale bar = 100 μm and 25 μm respectively) (n = 3). b Co-immunofluorescence of lumber ventral horn at × 40 magnification using UBQLN2 and TDP-43 antibodies (scale bar = 25 μm) (n = 3). c Co-immunofluorescence of lumbar ventral horn at × 40 magnification using SQSTM1/P62 and TDP-43 antibodies (scale bar = 25 μm) (n = 3). d Brain soluble-insoluble protein extracts from 8 months old non-transgenic (NTG, n = 3), UBQLN2^P497H (UBQ, n = 2), TDP-43^G348C (TDP, n = 3), and double-transgenic UBQLN2^P497H; TDP-43^G348C (UBQ; TDP, n = 2) mice. e Quantification of the TDP-43 levels vs GAPDH levels in the brain insoluble extracts. f Co-immunofluorescence of lumbar ventral horn at × 100 magnification using TDP-43 and phospho-TDP-43 antibodies (scale bar = 10 μm). g Total protein extracts from spinal cord of five 8 months old TDP-43^G348C [1–3, 7, 8] and five double-transgenic UBQLN2^P497H; hTDP-43^G348C mice [4–6, 9, 10]. h Quantification of the pTDP-43 levels vs GAPDH levels in the spinal cord. White arrow = TDP-43 inclusions (8 months), arrow head = UBQLN2 punctate signals (5 months)