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Table 1.

Examined human cases.

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Figure 1.

In vivo imaging of fibrillar tau aggregates in the retina of P301S mice.

A SLO examination of the mouse retina using a custom made mouse holder. B, C Pre-examination revealing spots of increased autofluorescence in the retinas of 5-month-old P301S and C57Bl/6 mice. D 24–48 hours after the injection of FSB, fluorescent cells were detected in the retinas of P301S mice but not of C57Bl/6 mice at 450 nm excitation (E). F, G No FSB-positive signals could be detected in the same imaging session when excited at 488 nm. H, I Example for the spectral discrimination of FSB-positive cells from spots of increased autofluorescence in the retina of a P301S mouse. H In vivo image obtained at 488 nm excitation showing dots of increased autofluorescence (AF, red arrows). I FSB-positive cells can only be excited at 450 nm (green arrows).

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Figure 2.

FSB labels cells containing hyperphosphorylated tau in the GCL of P301S mice.

A, B In vivo SLO-examination of a P301S mouse crossed with Thy1-YFPH. A Scanning at 488 nm excitation revealed only YFP-positive cells in a unique pattern (Striking cells are numbered 1–4). B In the same retinal area, FSB-positive cells (arrows) adjacent to YFP-positive cells became apparent when excited at 450 nm. C Whole mount preparation of the in vivo examined retina (A, B). D Magnification of the boxed area in B and C. The in vivo observed FSB-positive cells (B) could be counterstained with AT8 (arrows). Axons from RGCs containing hyperphosphorylated tau appear as red background. red, AT8; white, FSB; green, YFP. Scale bars: 50 µm.

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Figure 3.

Binding kinetics of FSB.

A 10 minutes after the systemic administration, no fluorescence of FSB was observed in SLO images obtained with 450 nm excitation. B Within retinal blood vessels, bright fluorescence appeared 60 min post-injection and was still present 12 hours post-injection (C). D 24 hours post-injection most of the fluorophore was cleared from the blood stream and distinct FSB-positive cells could be observed. E–G These FSB-positive cells were still present 48, 72 hours and even 1 month after the first injection of FSB. H New FSB-positive cells did not appear until a second FSB injection (arrow). p.i. post-injection.

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Figure 4.

In vivo long term imaging of FSB-positive cells displays the disease progression in P301S mice.

A–D Representative retina imaged between 2 and 5 months of age in an interval of 1 month. F–I Second retina imaged between 5 and 6.5 months of age in an interval of 2 weeks. A'–I' Enlarged images of the boxed areas in A and F demonstrating the appearance of new FSB-positive cells over the time (arrows). FSB was administrated systemically before each imaging session and images were acquired at 450 nm excitation. E, J Quantification of the increasing number of FSB-positive cells in the age from 2 to 5 months and 5 to 6.5 months. The curve displays the mean from 10 different retinas (n = 5 mice). Error bars show SD. **P<0.01; ***P<0.001.

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Figure 5.

Ex vivo analysis of FSB-positive cells.

A, B FSB-positive cells were counted in retinal whole mounts of P301S mice (red, AT8; white, FSB; green, YFP). C Quantification of FSB-positive cells of homozygous and heterozygous P301S mice in cohorts of different ages (n = 5–6). Each data point represents the number of FSB-positive cells in one retina normalized to the area. Shown are mean values ± SD. **P<0.01; ***P<0.001. D, E AT8-staining in the cortex of homozygous P301S mice at the age of 2 and 5 months. Scale bars: 1 mm (A), 100 µm (B, D, E).

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Figure 6.

Pathological alterations of tau and Aβ in the retinas of human AD and PSP patients.

A–C Post mortem retinas of AD and PSP patients showing AT8-positive cells in the INL (arrows) and stainings of the plexiform layers and GCL. B, B' Higher magnification image of individual AT8-positive cells in the INL. D Control retinas were not immunopositive for AT8. E–E'' AT8-positive cells could not be co-stained with FSB in AD and PSP retinas. F No 4G8-positive staining against Aβ was found in the retinas of AD patients. G In comparison, control brain slices of AD patients showed severe 4G8-positive Aβ-plaques. ONL, outer nuclear layer; OPL, outer plexiform layer; INL, inner nuclear layer; IPL, inner plexiform layer; GCL, ganglion cell layer. AD, Alzheimer's disease; PSP, progressive supranuclear palsy. Scale bars: 10 µm.

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