IndraLab

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USP19 activates ATXN3. 5 / 5
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"We next examined the effect of usp19 silencing on the protein levels of Atx3 100Q and Htt-N552 100Q, and observed that knockdown of USP19 significantly reduced the protein levels of Atx3 100Q (XREF_FIG) and Htt-N552 100Q (XREF_FIG) both in HEK 293T cells and in human retinal pigment epithelial (RPE1) cells (XREF_SUPPLEMENTARY)."
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"Beclin-1 stabilization is also promoted by USP19 and ataxin 3, which specifically removes K11- and K48-ubiquitin chain from Belcin-1, respectively [38, 39]."
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"The major finding of this work is that cytoplasmic USP19 can promote aggregation of the polyQ expanded Atx3 and Htt proteins by up-regulating their protein levels."
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"The regulatory function of USP19 was recently confirmed in a study demonstrating that USP19 interacts directly with chaperone Hsp90 and upregulates the aggregation of poly-Q containing the proteins Ataxin-3 and Huntingtin, which causes spinocerebellar ataxia type-3 and Huntington’s disease, respectively [90]."
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"Cytoplasmic Ubiquitin Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone."
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