IndraLab

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DNAJB1 inhibits ATXN3. 10 / 11
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"For example, HSP40 and HSP70 were reported to localize in the intranuclear aggregates formed by mutant ataxin-3 and that overexpression of HSP40 reduces aggregation of truncated and full-length Atx3 XREF_BIBR."
21625540
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"We conclude that exogenous DnaJ-1 suppresses the toxicity of pathogenic Atxn3 independently of lysine mutations.Thus far, we have observed mild but significant differences in the toxicity Atxn3(Q80) v[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
37865002
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"In support of this, it has been shown that overexpression of DNAJB1 restores the cellular degradation capacity [23] and suppresses aggregation of polyQ expanded ATXN3 [24]."
30086154
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"DNAJA1 effectively degrades huntington aggregates; DNAJB1 can degrade protein aggregates ataxin-3; DNAJB2 can inhibit the formation of huntington aggregates; DNAJB6 can inhibit the aggregation of Aβ 42 and α-synuclein; DNAJC5 can promote the release of TDP-43, τ protein, and α-synuclein into the extracellular space."
36581576
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"The validity of this approach was confirmed by recent data that the overexpression of human Ydj1 homologs HDJ-1 and HDJ-2 in mammalian cells suppressed the aggregation and toxicity of ataxin-3 with expanded polyglutamine tract [14]."
11102806
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"An overexpression of HSP40 and HDJ -2 suppressed ataxin-3 and ataxin-1 aggregation in vitro [XREF_BIBR, XREF_BIBR], but not in huntingtin exon 1 overexpressing cell lines [XREF_BIBR]."
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"In addition, upregulation of the molecular chaperones Hsp40 and Hsp70 powerfully suppressed neurodegeneration in Drosophila models of SCA1 and SCA3, respectively [47, 51]."
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"As shown in Figure 4B, co-expression of DnaJ-1 in adult fly neurons reduces the toxicity of both Atxn3 with a functional UbS1 and with UbS1*, independently of sex (Figure 4B)."
36733922
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"Over-expression of the Drosophila DNAJB1 homolog, dHDJ1, suppressed polyQ Htt- and ATXN3 dependent toxicity [XREF_BIBR, XREF_BIBR], and the effect of dHDJ1 on ATXN3 mediated toxicity was enhanced by over-expression with wildtype Hsp70 but inhibited by co-expression with a dominant negative mutant of Hsp70."
31552448
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"DNAJB1 was identified to suppress aggregate formation of ATXN3, but aggregation of the S256A mutant of ATXN3 could not be prevented by DNAJB1, it is still unclear whether DNAJB1 has preferential affinity for phosphorylated ATXN3."
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