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Fig. 11 | Acta Neuropathologica Communications

Fig. 11

From: Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

Fig. 11

a Electron micrographs of two layer 3 pyramidal neurons in the neocortex from a 15.5 month-old CGG159 KI mouse (159 repeats) showing the nucleoli (asterisk) and intranuclear inclusions (double asterisks) in each of the nuclei (nu). Inset “a” shows the nucleolus (asterisk) and a non-membrane bound electron-dense inclusion (double asterisks) of the left neuron. b Higher magnification of the adjacent region between the nucleolus (single asterisk) and intranuclear inclusion (double asterisks) from inset “a”. Note the granulo-filamentous ultrastructure of the inclusion material. c Electron micrograph of a protoplasmic astroglia in the neocortex of the same CGG KI mouse showing the marginal localization of the heterochromatin (arrowheads) and an intranuclear inclusion (arrow) within the nucleus (nu). d The inclusion in C shown at higher magnification consists of a predominantly granular material (double asterisks) surrounding some chromatin-like dense material (single asterisk) within the center. Scale bars: a, 10 μm; b, 1 μm; b, 5 μm; c, e, 0.2 μm; d, 2 μm. e Electron micrograph of a fibrous astroglia in the posterior hypothalamus of a Gfa2-CGG99 mouse with an intranuclear inclusion (double asterisk) within the nucleus (nu). Note the marginal chromatin localization (arrowheads) in the nucleus. f Higher magnification of the intranuclear inclusion (double asterisk) reveals an electron-dense structure inclusion body made up of predominantly granular material and filaments (small arrow). Scale bars: M 1 μm; N 0.2 μm. g Electron micrograph of a principal neuron in the posterior hypothalamus of a Gfa2-CGG99 mouse that shows the nucleolus (single asterisk) and an intranuclear inclusion (double asterisks) within the nucleus (nu). h Higher magnification of the adjacent regions of the nucleolus (single asterisk) and the intranuclear inclusion (double asterisk) in g which exhibit different ultrastructural features of the granular-filamentous material in the nucleolus versus the inclusion. Note the higher electron density of the nucleolus (pars granulosa and fibrosa) as compared with the more uniform appearing inclusion material. Scale bars: g: 2 μm; h: 0.2 μm. i Electron micrographs of intracytoplasmic inclusion body located within astrocytic processes in the posterior hypothalamus of a Gfa2-CGG99 mouse. The inclusions display an electron-dense core (double asterisks) and a lighter rim (single asterisk) which varied in size between inclusions (compare with inset i). Note the intermediate filaments within the cytoplasm (if) near the inclusion. j Higher magnification of the inclusion body in I presents an amorphous to granular material within the core and a granular-filamentous material within the rim. Note the intermediate filaments (if) - a characteristic feature of the astrocytic cytoplasm. k Intracytoplasmic inclusion body in the posterior hypothalamus exhibits a large electron-dense core surrounded by a thinner and less dense rim region. Note the mitochondria (M) in the adjacent cytoplasm of the astrocytic process. l Higher magnification of a portion from the inclusion body shows a linear-oriented filamentous material in the rim (asterisk) and a dense granular-filamentous material in the outer zone of the core (small arrows). Scale bars: i, i, 1 μm; k, 1 μm; j, l, 0.2 μm

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