IndraLab

Statements


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"Further studies indicate that ubiquitin-dependent activation of ataxin-3 at Lys-117 is important for its ability to reduce high molecular weight ubiquitinated species in cells."

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"AT3 is composed of a structured globular N-terminal domain, the Josephin domain (JD), which displays ubiquitin hydrolase activity, followed by a disordered C-terminal tail containing two ubiquitin interacting motifs (UIMs) and the polyQ stretch of variable length, whose expansion beyond a certain threshold triggers SCA3 [XREF_BIBR, XREF_BIBR]."

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"Further studies indicate that ubiquitin dependent activation of ataxin-3 at Lys 117 is important for its ability to reduce high molecular weight ubiquitinated species in cells."

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"For SCA 3, it is known that both normal and polyQ expanded ataxin-3 localize to mitochondria [XREF_BIBR] and that degradation of polyQ expanded ataxin-3 via the UPS is promoted by an ubiquitin ligase in the outer mitochondrial membrane called MITOL [XREF_BIBR]."

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"Cytoplasmic Ubiquitin Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone."