Quiet as a mouse: dissecting the molecular and genetic basis of hearing

被引:105
作者
Brown, Steve D. M. [1 ]
Hardisty-Hughes, Rachel E. [1 ]
Mburu, Philomena [1 ]
机构
[1] MRC, Mammalian Genet Unit, Didcot OX11 ORD, Oxon, England
基金
英国医学研究理事会;
关键词
D O I
10.1038/nrg2309
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Mouse genetics has made crucial contributions to the understanding of the molecular mechanisms of hearing. With the help of a plethora of mouse mutants, many of the key genes that are involved in the development and functioning of the auditory system have been elucidated. Mouse mutants continue to shed light on the genetic and physiological bases of human hearing impairment, including both early- and late-onset deafness. A combination of genetic and physiological studies of mouse mutant lines, allied to investigations into the protein networks of the stereocilia bundle in the inner ear, are identifying key complexes that are crucial for auditory function and for providing profound insights into the underlying causes of hearing loss.
引用
收藏
页码:277 / 290
页数:14
相关论文
共 105 条
[61]  
Mburu Philomena, 1997, Genes and Function, V1, P191
[62]   The very large G-protein-coupled receptor Vlgr1: A component of the ankle link complex required for the normal development of auditory hair bundles [J].
McGee, JoAnn ;
Goodyear, Richard J. ;
McMillan, D. Randy ;
Stauffer, Eric A. ;
Holt, Jeffrey R. ;
Locke, Kirsten G. ;
Birch, David G. ;
Legan, P. Kevin ;
White, Perrin C. ;
Walsh, Edward J. ;
Richardson, Guy P. .
JOURNAL OF NEUROSCIENCE, 2006, 26 (24) :6543-6553
[63]   An Fgf8 mutant allelic series generated by Cre- and Flp-mediated recombination [J].
Meyers, EN ;
Lewandoski, M ;
Martin, GR .
NATURE GENETICS, 1998, 18 (02) :136-141
[64]   Molecular characterization of the ankle-link complex in cochlear hair cells and its role in the hair bundle functioning [J].
Michalski, Nicolas ;
Michel, Vincent ;
Bahloul, Amel ;
Lefevre, Gaelle ;
Barral, Jeremie ;
Yagi, Hideshi ;
Chardenoux, Sebastien ;
Weil, Dominique ;
Martin, Pascal ;
Hardelin, Jean-Pierre ;
Sato, Makoto ;
Petit, Christine .
JOURNAL OF NEUROSCIENCE, 2007, 27 (24) :6478-6488
[65]  
MICHEL V, 2005, DEV BIOL, V23, P53
[66]   The deaf mouse mutant whirler suggests a role for whirlin in actin filament dynamics and stereocilia development [J].
Mogensen, Mette M. ;
Rzadzinska, Agnieszka ;
Steel, Karen R. .
CELL MOTILITY AND THE CYTOSKELETON, 2007, 64 (07) :496-508
[67]   Identification of Vangl2 and Scrb1 as planar polarity genes in mammals [J].
Montcouquiol, M ;
Rachel, RA ;
Lanford, PJ ;
Copeland, NG ;
Jenkins, NA ;
Kelley, MW .
NATURE, 2003, 423 (6936) :173-177
[68]   Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit [J].
Nelson, Rick F. ;
Glenn, Kevin A. ;
Zhang, Yuzhou ;
Wen, Hsiang ;
Knutson, Tina ;
Gouvion, Cynthia M. ;
Robinson, Barbara K. ;
Zhou, Zouping ;
Yang, Baoli ;
Smith, Richard J. H. ;
Paulson, Henry L. .
JOURNAL OF NEUROSCIENCE, 2007, 27 (19) :5163-5171
[69]   Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss [J].
Noben-Trauth, K ;
Zheng, QY ;
Johnson, KR .
NATURE GENETICS, 2003, 35 (01) :21-23
[70]   mdfw: A deafness susceptibility locus that interacts with deaf waddler (dfw) [J].
NobenTrauth, K ;
Zheng, QY ;
Johnson, KR ;
Nishina, PM .
GENOMICS, 1997, 44 (03) :266-272