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A Hearing and Vestibular Phenotyping Pipeline to Identify Mouse Mutants with Hearing Impairment

Overview
Journal Nat Protoc
Specialties Biology
Pathology
Science
Date 2010 Jan 9
PMID 20057387
Citations 68
Authors
Affiliations
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Abstract

We describe a protocol for the production of mice carrying N-ethyl-N-nitrosourea (ENU) mutations and their screening for auditory and vestibular phenotypes. In comparison with the procedures describing individual phenotyping tests, this protocol integrates a set of tests for the comprehensive determination of the causes of hearing loss. It comprises a primary screen of relatively simple auditory and vestibular tests. A variety of secondary phenotyping protocols are also described for further investigating the deaf and vestibular mutants identified in the primary screen. The screen can be applied to potentially thousands of mutant mice, produced either by ENU or other mutagenesis approaches. Primary screening protocols take no longer than a few minutes, apart from ABR testing which takes upto 3.5 h per mouse. These protocols have been applied for the identification of mouse models of human deafness and are a key component for investigating the genes and genetic pathways involved in hereditary deafness.

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References
1.
Hardisty-Hughes R, Tateossian H, Morse S, Romero M, Middleton A, Tymowska-Lalanne Z . A mutation in the F-box gene, Fbxo11, causes otitis media in the Jeff mouse. Hum Mol Genet. 2006; 15(22):3273-9. DOI: 10.1093/hmg/ddl403. View

2.
Thaung C, West K, Clark B, McKie L, Morgan J, Arnold K . Novel ENU-induced eye mutations in the mouse: models for human eye disease. Hum Mol Genet. 2002; 11(7):755-67. DOI: 10.1093/hmg/11.7.755. View

3.
Sharpe J, Ahlgren U, Perry P, Hill B, Ross A, Hecksher-Sorensen J . Optical projection tomography as a tool for 3D microscopy and gene expression studies. Science. 2002; 296(5567):541-5. DOI: 10.1126/science.1068206. View

4.
Zheng Q, Tong Y, Alagramam K, Yu H . Tympanometry assessment of 61 inbred strains of mice. Hear Res. 2007; 231(1-2):23-31. PMC: 2000814. DOI: 10.1016/j.heares.2007.05.011. View

5.
Inoue M, Sakuraba Y, Motegi H, Kubota N, Toki H, Matsui J . A series of maturity onset diabetes of the young, type 2 (MODY2) mouse models generated by a large-scale ENU mutagenesis program. Hum Mol Genet. 2004; 13(11):1147-57. DOI: 10.1093/hmg/ddh133. View