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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

Fig. 4

Localisation of NEAT1_1 outside paraspeckles in patient fibroblasts bearing FUS mutation. a FUS is predominantly nuclear in human patient fibroblasts bearing FUS P525L mutation. b Paraspeckle assembly is augmented in FUS P525L human fibroblasts. Paraspeckles were visualised by NEAT1_2 (3′ segment probe) RNA-FISH. *p < 0.05 (Mann-Whitney U-test). c Diffuse, non-paraspeckle distribution of NEAT1 in FUS P525L fibroblasts revealed using RNA-FISH with 5′ segment NEAT1 probe (total NEAT1). d NEAT1_1 is abnormally localised to nuclear speckles in FUS P525L fibroblasts. Representative images and quantification of the fraction of cells with speckle-localised NEAT1 are shown. Total NEAT1 (5′ segment probe) was used, and speckles were visualised by polyA+ RNA FISH. In b and d, numbers of cells analysed are indicated within bars. Scale bars, 10 μm

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