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. 2014 Nov 26:2:162.
doi: 10.1186/s40478-014-0162-2.

FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor/ataxia syndrome

Ronald A M Buijsen, Chantal Sellier, Lies-Anne W F M Severijnen, Mustapha Oulad-Abdelghani, Rob F M Verhagen, Robert F Berman, Nicolas Charlet-Berguerand, Rob Willemsen, Renate K Hukema  1
Affiliations

FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor/ataxia syndrome

Ronald A M Buijsen et al. Acta Neuropathol Commun. .

Abstract

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Electronic supplementary material: The online version of this article (doi:10.1186/s40478-014-0162-2) contains supplementary material, which is available to authorized users.

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Figures

Figure 1
Figure 1
9FM FMRpolyG-positive intranuclear inclusions in hippocampus, cerebellum and non-CNS tissues of a FXTAS patient. FMRpolyG-positive (9FM) intranuclear inclusions in a hippocampus, b cerebellum, c glomeruli and d distal tubule of the kidney, e zona glomerulosa and f zona reticularis of adrenal gland, g cardiomyocytes and h thyroid. All sections were immunostained with 9FM antibody and counterstained with hematoxylin. Scale bars represent 50 μm.
Figure 2
Figure 2
Colocalization of FMRpolyG (8FM) and ubiquitin in intranuclear inclusions in hippocampus, cerebellum and of non-CNS tissues of a FXTAS patient. Staining for ubiquitin (green), FMRpolyG (8FM; red) and DAPI (blue). Colocalization of ubiquitin and FMRpolyG (yellow) is seen in a hippocampus, b cerebellum, c kidney, d adrenal gland, e cardiomyocytes, and f thyroid; g quantification of inclusions containing ubiquitin and/or FMRpolyG (n = 100). Scale bars represent 10 μm.
See this image and copyright information in PMC

References

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