Ontology highlight
ABSTRACT:
SUBMITTER: Freeman S
PROVIDER: S-EPMC6726910 | biostudies-literature | 2019 Sep
REPOSITORIES: biostudies-literature
Freeman Stephen S Mateo Sánchez Susana S Pouyo Ronald R Van Lerberghe Pierre-Bernard PB Hanon Kevin K Thelen Nicolas N Thiry Marc M Morelli Giovanni G Van Hees Laura L Laguesse Sophie S Chariot Alain A Nguyen Laurent L Delacroix Laurence L Malgrange Brigitte B
EMBO reports 20190719 9
Protein homeostasis is essential to cell function, and a compromised ability to reduce the load of misfolded and aggregated proteins is linked to numerous age-related diseases, including hearing loss. Here, we show that altered proteostasis consequent to Elongator complex deficiency also impacts the proper development of the cochlea and results in deafness. In the absence of the catalytic subunit Elp3, differentiating spiral ganglion neurons display large aggresome-like structures and undergo ap ...[more]