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Pou3f4 deficiency causes defects in otic fibrocytes and stria vascularis by different mechanisms

 Mee Hyun Song  ;  Soo-Young Choi  ;  Ling Wu  ;  Se-Kyoung Oh  ;  Hee Keun Lee  ;  Dong Jin Lee  ;  Dae-Bo Shim  ;  Jae Young Choi  ;  Un-Kyung Kim  ;  Jinwoong Bok 
 Biochemical and Biophysical Research Communications, Vol.404(1) : 528-533, 2011 
Journal Title
 Biochemical and Biophysical Research Communications 
Issue Date
DFN3, the most prevalent X-linked hearing loss, is caused by mutations in the POU3F4 gene. Previous studies in Pou3f4 knockout mice suggest that defective otic fibrocytes in the spiral ligament of the cochlear lateral wall may underlie the hearing loss in DFN3. To better understand the pathological mechanisms of the DFN3 hearing loss, we analyzed inner ears of Pou3f4-deficient mice during development. Our results indicate that compartmentalization of the spiral ligament mesenchyme setting up boundaries for specific otic fibrocytes occurs normally in Pou3f4-deficient cochlea. However, differentiation of the compartmentalized mesenchyme into specific otic fibrocytes was blocked in the absence of Pou3f4 function. In addition, we found that stria vascularis in the cochlear lateral wall was also affected in Pou3f4-deficient cochlea. Unlike the otic fibrocytes, differentiation of stria vascularis was completed in the absence of Pou3f4 function, yet expression of Kir4.1 channels in the strial intermediate cells, essential for the sound transduction, was lost afterwards. These results suggest that Pou3f4 deficiency causes defects in both otic fibrocytes and stria vascularis at different developmental stages and by different pathological mechanisms, which may account for the progressive nature of DFN3 hearing loss.
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Yonsei Biomedical Research Center (연세의생명연구원)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Anatomy (해부학교실)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학교실)
Yonsei Authors
복진웅(Bok, Jin Woong) ; 송미현(Song, Mee Hyun) ; 심대보(Shim, Dae Bo) ; 오령(Wu, Ling) ; 최재영(Choi, Jae Young)
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