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Corrigendum Open Access | 10.1172/JCI194757

Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy

Rocio Bengoechea, Andrew R. Findlay, Ankan K. Bhadra, Hao Shao, Kevin C. Stein, Sara K. Pittman, Jil A.W. Daw, Jason E. Gestwicki, Heather L. True, and Conrad C. Weihl

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Published May 15, 2025 - More info

Published in Volume 135, Issue 10 on May 15, 2025
J Clin Invest. 2025;135(10):e194757. https://doi.org/10.1172/JCI194757.
© 2025 Bengoechea et al. This work is licensed under the Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
Published May 15, 2025 - Version history
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Related article:

Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy
Rocio Bengoechea, … , Heather L. True, Conrad C. Weihl
Rocio Bengoechea, … , Heather L. True, Conrad C. Weihl
Research Article Cell biology Muscle biology

Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy

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Abstract

Dominant mutations in the HSP70 cochaperone DNAJB6 cause a late-onset muscle disease termed limb-girdle muscular dystrophy type D1 (LGMDD1), which is characterized by protein aggregation and vacuolar myopathology. Disease mutations reside within the G/F domain of DNAJB6, but the molecular mechanisms underlying dysfunction are not well understood. Using yeast, cell culture, and mouse models of LGMDD1, we found that the toxicity associated with disease-associated DNAJB6 required its interaction with HSP70 and that abrogating this interaction genetically or with small molecules was protective. In skeletal muscle, DNAJB6 localizes to the Z-disc with HSP70. Whereas HSP70 normally diffused rapidly between the Z-disc and sarcoplasm, the rate of diffusion of HSP70 in LGMDD1 mouse muscle was diminished, probably because it had an unusual affinity for the Z-disc and mutant DNAJB6. Treating LGMDD1 mice with a small-molecule inhibitor of the DNAJ-HSP70 complex remobilized HSP70, improved strength, and corrected myopathology. These data support a model in which LGMDD1 mutations in DNAJB6 are a gain-of-function disease that is, counterintuitively, mediated via HSP70 binding. Thus, therapeutic approaches targeting HSP70-DNAJB6 may be effective in treating this inherited muscular dystrophy.

Authors

Rocio Bengoechea, Andrew R. Findlay, Ankan K. Bhadra, Hao Shao, Kevin C. Stein, Sara K. Pittman, Jil A.W. Daw, Jason E. Gestwicki, Heather L. True, Conrad C. Weihl

×

Original citation: J Clin Invest. 2020;130(8):4470–4485. https://doi.org/10.1172/JCI136167

Citation for this corrigendum: J Clin Invest. 2025;135(10):e194757. https://doi.org/10.1172/JCI194757

In Figure 2B of the original article, an incorrect image was included for the DNAJB6 WT sample, which was an inadvertent duplication and rotation of the image for the DNAJB6 F93L-H31Q sample. The corrected figure, based on the original source data, is provided below. The HTML and PDF versions of the paper have been updated.

Figure 2

The authors regret the error.

Footnotes

See the related article at Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy.

Version history
  • Version 1 (May 15, 2025): Electronic publication

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