西亚试剂：c-Ret–mediated hearing loss in mice with Hirschsprung disea

c-Ret–mediated hearing loss in mice with Hirschsprung disease
Nobutaka Ohgamia, Michiru Ida-Etoa, Takashi Shimotakeb, Naomi Sakashitac, Michihiko Soned, Tsutomu Nakashimad, Keiji Tabuchie, Tomofumi Hoshinoe, Atsuyoshi Shimadaf, Toyonori Tsuzukig, Masahiko Yamamotoh, Gen Sobuei, Mayumi Jijiwaj, Naoya Asaij, Akira Harae, Masahide Takahashij, and Masashi Katoa,1

A significantly increased risk for dominant sensorineural deafness in patients who have Hirschsprung disease (HSCR) caused by endothelin receptor type B and SOX10 has been reported. Despite the fact that c-RET is the most frequent causal gene of HSCR, it has not been determined whether impairments of c-Ret and c-RET cause congenital deafness in mice and humans. Here, we show that impaired phosphorylation of c-Ret at tyrosine 1062 causes HSCR-linked syndromic congenital deafness in c-Ret knockin (KI) mice. The deafness involves neurodegeneration of spiral ganglion neurons (SGNs) with not only impaired phosphorylation of Akt and NF-κB but decreased expression of calbindin D28k in inner ears. The congenital deafness involving neurodegeneration of SGNs in c-Ret KI mice was rescued by introducing constitutively activated RET. Taken together with our results for three patients with congenital deafness with c-RET–mediated severe HSCR, our results indicate that c-Ret and c-RET are a deafness-related molecule in mice and humans.

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