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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: Neuropathology of Beta-propeller protein associated neurodegeneration (BPAN): a new tauopathy

Fig. 2

Histological features in WDR45 mutation. In the substantia nigra there was severe loss of pigmented neurons with gliosis, axonal spheroids (a) and iron deposition (b). Axonal spheroids were highlighted using immunohistochemical staining for phosphorylated neurofilaments (c, arrow). There was also extensive iron deposition in the globus pallidus (d). In the frontal cortex there was mild neuronal loss with superficial spongiosis (e), phosphorylated tau accumulation was abundant in the form of threads and neurofibrillary tangles (f) and there were occasional balloon neurons. Fibrillar tau in neurofibrillary tangles was demonstrated by argyrophilia (h) and immunohistochemistry using AT100 confirmed the presence of paired helical filament tau (i). The 3-repeat (j) and 4-repeat (k) tau isoform specific antibodies illustrate the contribution of both groups of tau isoforms. a & e haematoxylin and eosin; b & d Perl’s stain; c SMI31; f AT8; g αB crystallin; h Gallyas silver impregnation; i AT100; j 3R tau; k 4R tau. Bar in a represents 200 μm in d & e, 80 μm in a, 40 μm in b, c, f, g & ik

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