A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging.
Atoh1 is a transcription factor that regulates neural development in multiple tissues and is conserved among species. Prior mouse models of Atoh1, though effective and important in the evolution of our understanding of the gene, have been limited by perinatal lethality. Here we describe a novel poin...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Public Library of Science (PLoS)
2013-01-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC3827170?pdf=render |
| _version_ | 1818035276105121792 |
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| author | Kianoush Sheykholeslami Vikrum Thimmappa Casey Nava Xiaohui Bai Heping Yu Tihua Zheng Zhaoqiang Zhang Sheng Li Li Shuqing Liu Qing Yin Zheng |
| author_facet | Kianoush Sheykholeslami Vikrum Thimmappa Casey Nava Xiaohui Bai Heping Yu Tihua Zheng Zhaoqiang Zhang Sheng Li Li Shuqing Liu Qing Yin Zheng |
| author_sort | Kianoush Sheykholeslami |
| collection | DOAJ |
| description | Atoh1 is a transcription factor that regulates neural development in multiple tissues and is conserved among species. Prior mouse models of Atoh1, though effective and important in the evolution of our understanding of the gene, have been limited by perinatal lethality. Here we describe a novel point mutation of Atoh1 (designated Atoh1(trhl) ) underlying a phenotype of trembling gait and hearing loss. Histology revealed inner ear hair cell loss and cerebellar atrophy. Auditory Brainstem Response (ABR) and Distortion Product Otoacoustic Emission (DPOAE) showed functional abnormalities in the ear. Normal lifespan and fecundity of Atoh1(trhl) mice provide a complementary model to facilitate elucidation of ATOH1 function in hearing,central nervous system and cancer biology. |
| first_indexed | 2024-12-10T06:52:29Z |
| format | Article |
| id | doaj.art-36c65d033882420aa2ad0d2dc4698618 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-10T06:52:29Z |
| publishDate | 2013-01-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-36c65d033882420aa2ad0d2dc46986182022-12-22T01:58:31ZengPublic Library of Science (PLoS)PLoS ONE1932-62032013-01-01811e7979110.1371/journal.pone.0079791A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging.Kianoush SheykholeslamiVikrum ThimmappaCasey NavaXiaohui BaiHeping YuTihua ZhengZhaoqiang ZhangSheng Li LiShuqing LiuQing Yin ZhengAtoh1 is a transcription factor that regulates neural development in multiple tissues and is conserved among species. Prior mouse models of Atoh1, though effective and important in the evolution of our understanding of the gene, have been limited by perinatal lethality. Here we describe a novel point mutation of Atoh1 (designated Atoh1(trhl) ) underlying a phenotype of trembling gait and hearing loss. Histology revealed inner ear hair cell loss and cerebellar atrophy. Auditory Brainstem Response (ABR) and Distortion Product Otoacoustic Emission (DPOAE) showed functional abnormalities in the ear. Normal lifespan and fecundity of Atoh1(trhl) mice provide a complementary model to facilitate elucidation of ATOH1 function in hearing,central nervous system and cancer biology.http://europepmc.org/articles/PMC3827170?pdf=render |
| spellingShingle | Kianoush Sheykholeslami Vikrum Thimmappa Casey Nava Xiaohui Bai Heping Yu Tihua Zheng Zhaoqiang Zhang Sheng Li Li Shuqing Liu Qing Yin Zheng A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. PLoS ONE |
| title | A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. |
| title_full | A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. |
| title_fullStr | A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. |
| title_full_unstemmed | A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. |
| title_short | A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. |
| title_sort | new mutation of the atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging |
| url | http://europepmc.org/articles/PMC3827170?pdf=render |
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