Knock-In Mice with Myo3a Y137C Mutation Displayed Progressive Hearing Loss and Hair Cell Degeneration in the Inner Ear
Joint Authors
Fu, Xiaolong
Zhang, Aizhen
Wen, Zongzhuang
Gao, Jiangang
Li, Peipei
Zhang, Guangkai
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-07-05
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract EN
Myo3a is expressed in cochlear hair cells and retinal cells and is responsible for human recessive hereditary nonsyndromic deafness (DFNB30).
To investigate the mechanism of DFNB30-type deafness, we established a mouse model of Myo3a kinase domain Y137C mutation by using CRISPR/Cas9 system.
No difference in hearing between 2-month-old Myo3a mutant mice and wild-type mice was observed.
The hearing threshold of the ≥6-month-old mutant mice was significantly elevated compared with that of the wild-type mice.
We observed degeneration in the inner ear hair cells of 6-month-old Myo3a mutant mice, and the degeneration became more severe at the age of 12 months.
We also found structural abnormality in the cochlear hair cell stereocilia.
Our results showed that Myo3a is essential for normal hearing by maintaining the intact structure of hair cell stereocilia, and the kinase domain plays a critical role in the normal functions of Myo3a.
This mouse line is an excellent model for studying DFNB30-type deafness in humans.
American Psychological Association (APA)
Li, Peipei& Wen, Zongzhuang& Zhang, Guangkai& Zhang, Aizhen& Fu, Xiaolong& Gao, Jiangang. 2018. Knock-In Mice with Myo3a Y137C Mutation Displayed Progressive Hearing Loss and Hair Cell Degeneration in the Inner Ear. Neural Plasticity،Vol. 2018, no. 2018, pp.1-10.
https://search.emarefa.net/detail/BIM-1210094
Modern Language Association (MLA)
Li, Peipei…[et al.]. Knock-In Mice with Myo3a Y137C Mutation Displayed Progressive Hearing Loss and Hair Cell Degeneration in the Inner Ear. Neural Plasticity No. 2018 (2018), pp.1-10.
https://search.emarefa.net/detail/BIM-1210094
American Medical Association (AMA)
Li, Peipei& Wen, Zongzhuang& Zhang, Guangkai& Zhang, Aizhen& Fu, Xiaolong& Gao, Jiangang. Knock-In Mice with Myo3a Y137C Mutation Displayed Progressive Hearing Loss and Hair Cell Degeneration in the Inner Ear. Neural Plasticity. 2018. Vol. 2018, no. 2018, pp.1-10.
https://search.emarefa.net/detail/BIM-1210094
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1210094