新規自然発症ミオシン6-nullマウスの表現型および発現解析
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概要
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In humans, hearing is a major factor in quality of life. Mouse models are important tools for the discovery of genes responsible for genetic hearing loss, often enabling analysis of the processes that regulate the onset of deafness in humans. Thus far, at least 400 deafness mutants have been discovered in laboratory mouse populations and used in the study of deafness. Here we report the discovery of a new spontaneous recessive Rinshoken shaker/waltzer (rsv) mutant derived from our in-house C57BL/6J stock, which exhibits circling and/or head-tossing behaviour and complete lack of auditory brain response to any sound pressure. The hearing and balance phenotypes are associated with structural defects, in particular, disorganisation and fusion of stereocilia in the inner ear hair cells. Two sets of intersubspecific N2 mice were generated for the positional cloning of the rsv mutation. The mutant locus was mapped to a 4.8-Mb region of chromosome 9, which contains myosin VI (Myo6), a gene responsible for deafness in humans and Snells waltzer mutation in mice. The rsv mutant showed reduced expressions of Myo6 mRNA and MYO6 protein in the inner ear. Moreover, no immunoreactivity was observed in the cochlear and vestibular hair cells in the rsv mutant mice. We sequenced the genomic region (30,154 bp) of Myo6, including all coding exons, a non-coding exon, UTRs and the Myo6 promoter; however, no mutation was discovered in these regions. We therefore speculate that loss of MYO6 expression might cause shaker/waltzer behaviour and deafness in the rsv mutant; also, loss of MYO6 expression might be the result of mutations in an unidentified regulatory region(s) of the gene.
- 社団法人 日本実験動物学会の論文
- 2010-01-01
著者
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望月 英志
Department of Bioindustry, Tokyo University of Agriculture
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奥村 和弘
Department of Bioindustry, Tokyo University of Agriculture
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石川 昌志
Department of Bioindustry, Tokyo University of Agriculture
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YOSHIMOTO Sachi
Department of Bioindustry, Tokyo University of Agriculture
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YAMAGUCHI Junya
Department of Bioindustry, Tokyo University of Agriculture
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WADA Kenta
Department of Bioindustry, Tokyo University of Agriculture
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USHIKI Tatsuo
Department of Anatomy, Graduate School of Medical and Dental Sciences, Niigata University
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TOKANO Hisashi
Department of Otolaryngology, Tokyo Medical and Dental University
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ISHII Rie
Department of Laboratory Animal Science, The Tokyo Metropolitan Institute of Medical Science
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TAYA Choji
Department of Laboratory Animal Science, The Tokyo Metropolitan Institute of Medical Science
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Ishii Rie
Department Of Chemistry Toho University
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Yonekawa Hiromichi
Dep. Of Lab. Animal Sci. The Tokyo Metropolitan Inst. Of Medical Sci.
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Shitara Hiroshi
Department Of Laboratory Animal Science The Tokyo Metropolitan Institute Of Medical Science
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Yamaguchi Junya
Department Of Advanced Energy Engineering Science Interdisciplinary Graduate School Of Engineering S
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Kikkawa Y
Tokyo Metropolitan Inst. Medical Sci. Tokyo Jpn
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MOCHIZUKI Eiji
Department of Bioindustry, Tokyo University of Agriculture
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Yonekawa Hiromichi
Department Of Animal Science The Tokyo Metropolitan Institute Of Medical Science
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Kitamura Ken
Department Of Clinical Pharmacology School Of Medicine Tokai University
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Ishikawa Masashi
Department Of Applied Chemistry And Chemical Engineering Faculty Of Engineering Yamaguchi University
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Okumura Kazuhiro
Department Of Urology Faculty Of Medicine Kyoto University
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Tokano Hisashi
Department Of Otolaryngology Tokyo Medical And Dental University
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Kikkawa Yoshiaki
Department Of Bioindustry Tokyo University Of Agriculture
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Ushiki Tatsuo
Department Of Anatomy And Histology Faculty Of Medicine Niigata University
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Yamaguchi Junya
Department Of Bioindustry Tokyo University Of Agriculture
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Ishii Rie
Department Of Laboratory Animal Science The Tokyo Metropolitan Institute Of Medical Science
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Seki Yuta
Department Of Bioindustry Tokyo University Of Agriculture
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Taya Choji
Department Of Laboratory Animal Science The Tokyo Metropolitan Institute Of Medical Science
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Mochizuki Eiji
Department Of Bioindustry Tokyo University Of Agriculture
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Yokohama Michinari
Department Of Bioindustry Tokyo University Of Agriculture
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Wada Kenta
Department Of Bioindustry Tokyo University Of Agriculture
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Ushiki Tatsuo
Department Of Anatomy Graduate School Of Medical And Dental Sciences Niigata University
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Ishikawa Masashi
Department Of Bioindustry Tokyo University Of Agriculture
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Hirata Shuji
Department Of Laboratory Animal Science The Tokyo Metropolitan Institute Of Medical Science
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Yoshimoto Sachi
Department Of Bioindustry Tokyo University Of Agriculture
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Okumura Kazuhiro
Department Of Bioindustry Tokyo University Of Agriculture
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Ishii Rie
Department Of Analytical Chemistry Faculty Of Pharmaceutical Sciences Hoshi University
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Okumura Kazuhiro
Department Of Bacteriology Okayama University Graduate School Of Medicine Dentistry And Pharmaceutical Sciences
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TAYA Choji
Department of Animal Science The tokyo Metropolitan Institute of Medical Science
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