Article
The HSP70 co-chaperone DNAJC14 targets misfolded pendrin for unconventional protein secretion.
Nature communications - 25 Apr 2016
Jung Jinsei, Kim Jiyoon, Roh Shin Hye, Jun Ikhyun, Sampson Robert D, Gee Heon Yung, Choi Jae Young, Lee Min Goo
Abstract excerpt
Mutations in SLC26A4, which encodes pendrin, are responsible for hearing loss with an enlarged vestibular aqueduct and Pendred syndrome. The most prevalent mutation in East Asia is p.H723R (His723Arg), which leads to defects in protein folding and cell-surface expression. Here we show that H723R-pendrin can be rescued to the cell surface by an HSP70 co-chaperone DNAJC14-dependent unconventional trafficking...
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