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. 2013 Mar;125(3):463-5.
doi: 10.1007/s00401-013-1089-6. Epub 2013 Feb 3.

Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid

Eileen H Bigio, Jane Y Wu, Han-Xiang Deng, Esther N Bit-Ivan, Qinwen Mao, Rakhee Ganti, Melanie Peterson, Nailah Siddique, Changiz Geula, Teepu Siddique, Marsel Mesulam

Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid

Eileen H Bigio et al. Acta Neuropathol. 2013 Mar.
No abstract available

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Figures

Fig. 1
Fig. 1
Superficial frontal cortex, FTLD-TDP type A (1a). Numerous thioflavin-S positive c-shaped and annular NCIs and a few DNs. Inset: thioflavin-S positive NII. Thioflavin-S, 400x. Confocal microscopy (1b). Same case as Fig. 1a, with thioflavin-S (green, left), TDP-43 (red, middle), and overlay (yellow, right) showing co-localization in NCIs (arrows). Fluorescent confocal microscopy, thioflavin-S and TDP-43 immunohistochemistry. Frontal cortex (left) and dentate gyrus, FTLD-MND type B (1c). Granular thioflavin-S positive cortical CIs. Dentate gyrus CIs are both granular (top) and dense, c-shaped/perinuclear (middle). Rare NIIs were seen in type B cases (bottom) in only one case. Thioflavin-S, 600x. Frontal cortex, FTLD-TDP type C (1d). Numerous thioflavin-S positive thick, long DNs in cortex. Inset: several dense CIs, dentate gyrus. Thioflavin-S, 400x.
Fig. 1
Fig. 1
Superficial frontal cortex, FTLD-TDP type A (1a). Numerous thioflavin-S positive c-shaped and annular NCIs and a few DNs. Inset: thioflavin-S positive NII. Thioflavin-S, 400x. Confocal microscopy (1b). Same case as Fig. 1a, with thioflavin-S (green, left), TDP-43 (red, middle), and overlay (yellow, right) showing co-localization in NCIs (arrows). Fluorescent confocal microscopy, thioflavin-S and TDP-43 immunohistochemistry. Frontal cortex (left) and dentate gyrus, FTLD-MND type B (1c). Granular thioflavin-S positive cortical CIs. Dentate gyrus CIs are both granular (top) and dense, c-shaped/perinuclear (middle). Rare NIIs were seen in type B cases (bottom) in only one case. Thioflavin-S, 600x. Frontal cortex, FTLD-TDP type C (1d). Numerous thioflavin-S positive thick, long DNs in cortex. Inset: several dense CIs, dentate gyrus. Thioflavin-S, 400x.
Fig. 1
Fig. 1
Superficial frontal cortex, FTLD-TDP type A (1a). Numerous thioflavin-S positive c-shaped and annular NCIs and a few DNs. Inset: thioflavin-S positive NII. Thioflavin-S, 400x. Confocal microscopy (1b). Same case as Fig. 1a, with thioflavin-S (green, left), TDP-43 (red, middle), and overlay (yellow, right) showing co-localization in NCIs (arrows). Fluorescent confocal microscopy, thioflavin-S and TDP-43 immunohistochemistry. Frontal cortex (left) and dentate gyrus, FTLD-MND type B (1c). Granular thioflavin-S positive cortical CIs. Dentate gyrus CIs are both granular (top) and dense, c-shaped/perinuclear (middle). Rare NIIs were seen in type B cases (bottom) in only one case. Thioflavin-S, 600x. Frontal cortex, FTLD-TDP type C (1d). Numerous thioflavin-S positive thick, long DNs in cortex. Inset: several dense CIs, dentate gyrus. Thioflavin-S, 400x.
Fig. 1
Fig. 1
Superficial frontal cortex, FTLD-TDP type A (1a). Numerous thioflavin-S positive c-shaped and annular NCIs and a few DNs. Inset: thioflavin-S positive NII. Thioflavin-S, 400x. Confocal microscopy (1b). Same case as Fig. 1a, with thioflavin-S (green, left), TDP-43 (red, middle), and overlay (yellow, right) showing co-localization in NCIs (arrows). Fluorescent confocal microscopy, thioflavin-S and TDP-43 immunohistochemistry. Frontal cortex (left) and dentate gyrus, FTLD-MND type B (1c). Granular thioflavin-S positive cortical CIs. Dentate gyrus CIs are both granular (top) and dense, c-shaped/perinuclear (middle). Rare NIIs were seen in type B cases (bottom) in only one case. Thioflavin-S, 600x. Frontal cortex, FTLD-TDP type C (1d). Numerous thioflavin-S positive thick, long DNs in cortex. Inset: several dense CIs, dentate gyrus. Thioflavin-S, 400x.
Fig. 2
Fig. 2
Motor cortex, FTLD-FUS (BIBD) (2a). Large cytoplasmic upper motor neuron inclusion on left, consistent with basophilic inclusion body (BIB), negative with thioflavin-S. BIB on right immunolabeled with FUS for comparison. Thioflavin-S stain and FUS immunohistochemistry, 600x. Granular NCI in dentate gyrus, FTLD-FUS (aFTLD-U) (2b). Thioflavin-S, 600x.
Fig. 2
Fig. 2
Motor cortex, FTLD-FUS (BIBD) (2a). Large cytoplasmic upper motor neuron inclusion on left, consistent with basophilic inclusion body (BIB), negative with thioflavin-S. BIB on right immunolabeled with FUS for comparison. Thioflavin-S stain and FUS immunohistochemistry, 600x. Granular NCI in dentate gyrus, FTLD-FUS (aFTLD-U) (2b). Thioflavin-S, 600x.
Fig. 3
Fig. 3
Lumbar anterior horn, ALS. Lower motor neurons with LLB (left) and SLIs (middle and right). Scattered shrunken neurons nearby (middle and right images) contain compressed SLIs. Thioflavin-S, 600x.
Fig. 4
Fig. 4
Superficial frontal cortex, FTLD-TDP type A. Exuberant thioflavin-S positive astrocytosis. Thioflavin-S, 200x.
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References

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