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ATXN3 activates localization. 7 / 7
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"The main hallmark of SCA3 is the expansion of a polyglutamine tract located in the C-terminal of Ataxin-3 (or ATXN3) protein, that triggers the mis-localization and toxic aggregation of ATXN3 in neuronal cells."
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"For instance, phosphorylation or phosphomimetic mutations of S12 or S256 in ataxin-3 showed protective effects, by reducing toxic aggregation of the polyQ-expanded protein in vitro or in vivo (Fei et al., 2007; Matos et al., 2016), whereas casein kinase 2 (CK2)-associated phosphorylation of ataxin-3 triggered a detrimental nuclear localization and aggregation (Mueller et al., 2009)."
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"Mapping studies showed that two regions of Atx3, the Josephin domain and the C-terminus, regulated heat shock induced nuclear localization."
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"Coiled-Coil Structures of SCA3 polyQ Proteins Promote Their Nuclear Localization."
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"More evidence is needed to establish the physiological role of USP10 phosphorylation in tumorigenesis.In the case of ATX3, phosphorylation at Ser340 and Ser352 by CK2 enhances its nuclear localization, aggregation, and stability, processes that play a major role in the development of spinocerebellar ataxia-3."
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"d Spinocerebellar ataxia type 3 (SCA3): CK2 associates to and phosphorylates ataxin-3, thus promoting its nuclear localization and stabilization, and enhancing the formation of inclusions."
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"The product of this gene, ataxin-3, associates to and is phosphorylated by CK2 175 (Fig. 3d ), which induces its nuclear localization and stabilization, and enhances the formation of inclusions."
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