Autophagy is essential for hearing in mice

Chisato Fujimoto, Shinichi Iwasaki, Shinji Urata, Hideaki Morishita, Yuriko Sakamaki, Masato Fujioka, Kenji Kondo, Noboru Mizushima, Tatsuya Yamasoba

Research output: Contribution to journalArticlepeer-review

20 Scopus citations


Hearing loss is the most frequent sensory disorder in humans. Auditory hair cells (HCs) are postmitotic at late-embryonic differentiation and postnatal stages, and their damage is the major cause of hearing loss. There is no measurable HC regeneration in the mammalian cochlea, and the maintenance of cell function is crucial for preservation of hearing. Here we generated mice deficient in autophagy-related 5 (Atg5), a gene essential for autophagy, in the HCs to investigate the effect of basal autophagy on hearing acuity. Deletion of Atg5 resulted in HC degeneration and profound congenital hearing loss. In autophagy-deficient HCs, polyubiquitinated proteins and p62/SQSTM1, an autophagy substrate, accumulated as inclusion bodies during the first postnatal week, and these aggregates increased in number. These findings revealed that basal autophagy has an important role in maintenance of HC morphology and hearing acuity.

Original languageEnglish (US)
Article numbere2780
JournalCell Death and Disease
Issue number5
StatePublished - May 11 2017
Externally publishedYes

ASJC Scopus subject areas

  • Immunology
  • Cellular and Molecular Neuroscience
  • Cell Biology
  • Cancer Research


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