Sonic Hedgehog信号通路与内耳发育调控

doi:10.3724/SP.J.1005.2013.01058

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HEREDITAS ›› 2013, Vol. 35 ›› Issue (9): 1058-1064.doi: 10.3724/SP.J.1005.2013.01058

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Sonic Hedgehog signaling pathway and regulation of inner ear de-velopment

CHEN Zhi-Qiang¹, HAN Xin-Huan², CAO Xin²

1. The First Clinical Medical College, Nanjing Medical University, Nanjing 210029, China; 2. School of Basic Medicine, Nanjing Medical University, Nanjing 210029, China

Received:2013-04-22 Revised:2013-06-24 Online:2013-09-20 Published:2013-09-25

Abstract

Abstract:

During inner ear development, Sonic Hedgehog (Shh) signaling pathway is involved in the ventral otic identity, cell fate determination of statoacoustic ganglion neurons and hair cell development. Shh protein, secreted from floor plate, antagonizes Wnt protein from roof plate, which refines and maintains dorsoventral axial patterning in the ear. Shh, served as a mitogen during neurogenesis, directly promotes the development of spiral ganglion neuron. After Shh signaling pathway is activated, Ngn1 is freed from Tbx1 repression. As a result, Shh indirectly upregulates the expression of Ngn1, thus regulating neurogenic patterning of inner ear. In addition, Shh regulates the differentiation of hair cells by influencing cell cycle of the progenitor cells located in the cochlea. The basal-to-apical wave of Shh decline ensures the normal devel-opment pattern of hair cells. It is confirmed by a quantity of researches conducted in both animals and patients with heredi-tary hearing impairment that abnormal Shh signaling results in aberrant transcription of target genes, disturbance of the proper development of inner ear, and human hearing impairment. In humans, diseases accompanied by hearing disorders caused by abnormal Shh signaling include Greig cephalopolysyndactyly syndrome (GCPS), Pallister-Hall syndrome (PHS), Waardenburg syndrome (WS) and medulloblastoma, etc. This review would provide a theoretical basis for further study of molecular mechanisms and clinical use of inner ear development.

Key words: Sonic Hedgehog signaling, development, inner ear, hearing impairment

CHEN Zhi-Qiang HAN Xin-Huan CAO Xin. Sonic Hedgehog signaling pathway and regulation of inner ear de-velopment[J]. HEREDITAS, 2013, 35(9): 1058-1064.

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