Bcl11b Heterozygosity Leads to Age-Related Hearing Loss and Degeneration of Outer Hair Cells of the Mouse Cochlea

被引：6

作者：

Okumura, Hitoshi ^{[1
,2
]}

Miyasaka, Yuki ^{[1
]}

Morita, Yuka ^{[2
]}

Nomura, Tomoyuki ^{[2
]}

Mishima, Yukio ^{[1
]}

Takahashi, Sugata ^{[2
]}

Kominami, Ryo ^{[1
]}

机构：

[1] Niigata Univ, Grad Sch Med & Dent Sci, Dept Mol Genet, Niigata 9518510, Japan

[2] Niigata Univ, Grad Sch Med & Dent Sci, Dept Otorhinolaryngol, Niigata 9518510, Japan

来源：

EXPERIMENTAL ANIMALS | 2011年 / 60卷 / 04期

关键词：

BCL11B/CTIP2; Corti organ; hair cell; hearing loss; presbycusis; USHER-SYNDROME; CADHERIN GENE; CDH23; MUTATION; MICE; SUSCEPTIBILITY; ASSOCIATION; PROMOTER; WALTZER; STRAINS;

D O I：

10.1538/expanim.60.355

中图分类号：

S85 [动物医学（兽医学）];

学科分类号：

0906 ;

摘要：

BCL11B/CTIP2 zinc-finger transcription factor is expressed in various types of cells in many different tissues. This study showed that BCL11B is expressed in the nucleus of the outer hair cells of the mouse cochlea, degeneration of which is known to cause deafness and presbycusis or age-related hearing loss (AHL). We tested whether or not Bcl11b heterozygosity would affect AHL in mice. Analysis of auditory brainstem responses revealed AHL in Bcl11b(+/-) heterozygous, but not wild-type, mice, which was evident as early as 3 months after birth. Histological abnormalities were observed in the outer hair cells of the Bcl11b(+/-) mice at 6 months of age with hearing loss. These results suggest that the AHL observed in Bcl11b(+/-) mice is the result of impairment of the outer hair cells and that BCL11B activity is required for the maintenance of outer hair cells and normal hearing.

引用

页码：355 / 361

页数：7

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