Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy

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...

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Publicado en:J Clin Invest
Autores principales: Bengoechea, Rocio, Findlay, Andrew R., Bhadra, Ankan K., Shao, Hao, Stein, Kevin C., Pittman, Sara K., Daw, Jil A.W., Gestwicki, Jason E., True, Heather L., Weihl, Conrad C.
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Clinical Investigation 2020
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Research Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC7410071/
https://ncbi.nlm.nih.gov/pubmed/32427588
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI136167
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