Fig. 2

Distribution of ATXN2 in normal and FTLD-TDP human brain. Immunohistochemical labeling of normal control brain revealed the widespread distribution of ATXN2 in the neurons in the neocortex (a, c), limbic system, basal ganglia, cerebellum, midbrain, and anterior horn. a Low magnification image of the temporal neocortex showed neuronal ATXN2-immunoreactivity in II–VI laminae. c High magnification image of the III lamina. This image showed moderate neuronal ATXN2-immunoreactivity as a diffuse or granular appearance in perikarya and proximal apical and basal dendrites. The neuronal nuclei were mostly immunonegative. e Low magnification image of the hippocampal CA1-4 subfields and dentate gyrus, which showed the widespread distribution of neuronal ATXN2-immunoreactivity with less expression in the CA1 region. f High magnification image of the hippocampal CA3 showed strong ATXN2-immunoreactivity along with striated or dot appearances in the neuropil. g Mid magnification image of the striatum. The ATXN2-immunoreactivity in large interneurons (arrows and inset) was stronger than that in medium-size spiny neurons. h High magnification image of the Purkinje cells presenting strong ATXN2-immunoreactivity. i High magnification image of the substantia nigra pars compacta showing strong neuronal ATXN2-immunoreactivity. The neuropil was also immunolabeled as striated or dot appearances. j High magnification image of the anterior horn cells showed strong ATXN2-immunoreactivity. In the FTLD-TDP brain, the ATXN2-immunoreactivity was markedly decreased (b, d). b Low magnification image of the upper layer of the temporal neocortex showing the reduction of ATXN2-immunoreactivity in the pyramidal neurons, compared with that in controls shown in a. d High magnification image of the remaining neurons in the III lamina showed that the intracellular distribution of ATXN2 was not altered in diseased brains. The scale bars included in each image represent 50 µm (c, d, f, h, i, j), 100 µm (g), and 200 µm (a, b, e)