Current Gene Therapy

Ignacio Anegon
Director INSERM UMR 1064-Center for Research in Transplantation and Immunology
CHU de Nantes. 30, boulevard


Atoh1: Landscape for Inner Ear Cell Regeneration

Author(s): Ren Hongmiao, Liu Wei, Hu Bing, Ding Da Xiong and Ren Jihao

Affiliation: Department of Otolaryngology Head and Neck Surgery, the Second Xiangya Hospital, Central South University, No.139 Middle Renmin Road, Changsha, Hunan 410011, P.R. China.


Hearing impairment is primarily attributed to inner ear hair cell (HC) defects that subsequently lead to spiral ganglion neuron (SGN) loss. The HC loss cannot be self-repaired because of the HCs’ limited capacity to regenerate in mammals. Atoh1, also known as Math1, Hath1, and Cath1, is a proneural basic helix–loop–helix (bHLH) transcription factor that played a major role in HC differentiation. Atoh1 activity at various developmental stages can sufficiently drive HC differentiation in the cochlea. Recent issues of a certain publication have identified that Atoh1 is essential for inner ear development, such as cell growth, morphogenesis, differentiation, cellular maintenance, and survival. We summarize the new findings in Atoh1 research and identify the mechanisms underlying the role of Atoh1 in HC regeneration to launch the future of Atoh1 therapy.

Keywords: Atoh1, hair cell regeneration, hearing impairment, Notch signaling pathway, spiral ganglia neuron.

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Article Details

Page: [101 - 111]
Pages: 11
DOI: 10.2174/1566523214666140310143407