The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity.

Increased eye degeneration due to the downregulation HSP67Bc was found to correlate with a small but significant increase in the high molecular weight (MW) species typically formed on expression of mutant TDP-43-NLS (as previously described ( 32 )) and also with a highly significant accumulation of endogenous (poly)ubiquitinated proteins ( Figure 6B ).

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