Figure 1

Morphology of FUS inclusions in aFTLD-U and late-onset BIBD. Images of representative FUS inclusions from (A-G) aFTLD-U and (H-M) BIBD cases are shown. FUS IHC of aFTLD-U revealed various aggregates including (A) compact round NCIs in the dentate gyrus of the hippocampus, (B) tangle-like inclusions in frontal neocortex, (C) a conglomerate aggregate in a substantia nigra neuron, (D) perinuclear crescentic inclusions in frontal neocortex, (E) numerous vermiform NIIs in the medullary olive nucleus, (F) a ring shaped NII in a cerebellar dentate nucleus neuron and (G) a swollen DN in the entorhinal cortex. H&E stain of BIBD cases revealed basophilic inclusions including aggregates involving (H) the cerebellar dentate nucleus and (I) the primary motor cortex. Cresyl violet stain also stained BIBD inclusions including (J) an aggregate within a spinal motor neuron. FUS immunohistochemistry of BIBD cases revealed NCIs of various morphologies including (K) conglomerate aggregates within a substantia nigra neuron and round NCIs involving the (L) dentate nucleus of the cerebellum and (M) pontine nuclei. Scale bars for (A) 20 μm, (B-D, F-G) 10 μm, (E) 50 μm, (H-K) 10 μm and (L-M) 200 μm are shown.