Truncated HSPB1 causes axonal neuropathy and impairs tolerance to unfolded protein stress

Conclusion Our results suggest that truncation of the non-conserved C-terminus impairs the function of HSPB1 in cellular stress response. General significance sHSPs have important roles in prevention of protein aggregates that induce toxicity. We showed that C-terminal part of HSPB1 is critical for tolerance of unfolded protein stress, and when lacking causes axonal neuropathy in patients. Graphical abstract
Source: BBA Clinical - Category: Biochemistry Source Type: research