TY  - JOUR
TI  - Down-regulation of msrb3 and destruction of normal auditory system development through hair cell apoptosis in zebrafish
AU  - Shen, Xiaofang
AU  - Liu, Fei
AU  - Wang, Yingzhi
AU  - Wang, Huijun
AU  - Ma, Jing
AU  - Xia, Wenjun
AU  - Zhang, Jin
AU  - Jiang, Nan
AU  - Sun, Shaoyang
AU  - Wang, Xu
AU  - Ma, Duan
T2  - The International Journal of Developmental Biology
AB  - Hearing defects can significantly influence quality of life for those who experience them. At this time, 177 deafness genes have been cloned, including 134 non-syndromic hearing-loss genes. The methionine sulfoxide reductase B3 (Ahmed et al., 2011) gene (also called DFNB74) is one such newly discovered hearing-loss gene. Within this gene c.265 T>G and c.55 T>C mutations are associated with autosomal recessive hearing loss. However, the biological role and mechanism underlying how it contributes to deafness is unclear. Thus, to better understand this mutation, we designed splicing morpholinos for the purpose of down-regulating msrb3 in zebrafish. Morphants exhibited small, tiny, fused, or misplaced otoliths and abnormal numbers of otoliths. Down-regulation of msrb3 also caused shorter, thinner, and more crowded cilia. Furthermore, L1-8 neuromasts were reduced and disordered in the lateral line system; hair cells in each neuromast underwent apoptosis. Co-injection with human MSRB3 mRNA partially rescued auditory system defects, but mutant MSRB3 mRNA could not. Thus, msrb3 is instrumental for auditory system development in zebrafish and MSRB3-related deafness may be caused by promotion of hair cell apoptosis.
PY  - 2015
DO  - 10.1387/ijdb.140200md
VL  - 59
IS  - 4-5-6
SP  - 195
EP  - 203
J2  - Int. J. Dev. Biol.
LA  - en
SN  - 0214-6282
SN  - 1696-3547
UR  - https://ijdb.ehu.eus/article/140200md
Y2  - 2024/11/21/14:25:42
ER  -