Regular ArticleImmunodetection of Poly(A) Binding Protein II in the Cell Nucleus
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PABPN1 aggregation is driven by Ala expansion and poly(A)-RNA binding, leading to CFIm25 sequestration that impairs alternative polyadenylation
2023, Journal of Biological ChemistryRNA-Based Therapy Utilizing Oculopharyngeal Muscular Dystrophy Transcript Knockdown and Replacement
2019, Molecular Therapy Nucleic AcidsCitation Excerpt :Wild-type PABPN1 is an abundant nuclear protein, with widespread staining in the nucleoplasm as shown in C2C12 transfected with wild-type PABPN1-GFP (Figure 6A). It is mostly concentrated in discrete nuclear domains called speckles38–40 (Figure 6A). We examined the expression of opt-PABPN1-GFP and opt-PABPN1-Dsred using the fluorescent microscope.
Nuclear speckles are involved in nuclear aggregation of PABPN1 and in the pathophysiology of oculopharyngeal muscular dystrophy
2012, Neurobiology of DiseaseCitation Excerpt :The immunolabeling of the amorphous dense material indicates the existence of an unstructured fraction of PABPN1 that is not assembled into tubular filaments. Since both endogenous wild type PABPN1 and the fusion protein GFP-PABPN1wt co-localize with the splicing factor SC35 in nuclear speckles of human HeLa, A549-tTA and U2OS cells (Calado et al., 2000; Krause et al., 1994; Raz et al., 2011; Sasseville et al., 2006), we investigated whether this co-localization is preserved in myonuclei from both control and OPMD patients, and the possible spatial relationship between INIs and nuclear speckles. Co-staining for TMG-cap of snRNAs, a component of spliceosomal snRNPs that serves as a marker of nuclear speckles (Lamond and Spector, 2003), and PABPN1 in myonuclei from human controls revealed that both molecular components concentrate and co-localize in typical nuclear speckles, with a weaker signal apparent throughout the nucleoplasm excluding the nucleolus (Figs. 2A–C).
PABPN1 polyalanine tract deletion and long expansions modify its aggregation pattern and expression
2008, Experimental Cell Research