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...

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Autores principales: Sheykholeslami, Kianoush, Thimmappa, Vikrum, Nava, Casey, Bai, Xiaohui, Yu, Heping, Zheng, Tihua, Zhang, Zhaoqiang, Li, Sheng Li, Liu, Shuqing, Zheng, Qing Yin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3827170/
https://www.ncbi.nlm.nih.gov/pubmed/24265785
http://dx.doi.org/10.1371/journal.pone.0079791
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author Sheykholeslami, Kianoush
Thimmappa, Vikrum
Nava, Casey
Bai, Xiaohui
Yu, Heping
Zheng, Tihua
Zhang, Zhaoqiang
Li, Sheng Li
Liu, Shuqing
Zheng, Qing Yin
author_facet Sheykholeslami, Kianoush
Thimmappa, Vikrum
Nava, Casey
Bai, Xiaohui
Yu, Heping
Zheng, Tihua
Zhang, Zhaoqiang
Li, Sheng Li
Liu, Shuqing
Zheng, Qing Yin
author_sort Sheykholeslami, Kianoush
collection PubMed
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.
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spelling pubmed-38271702013-11-21 A New Mutation of the Atoh1 Gene in Mice with Normal Life Span Allows Analysis of Inner Ear and Cerebellar Phenotype in Aging Sheykholeslami, Kianoush Thimmappa, Vikrum Nava, Casey Bai, Xiaohui Yu, Heping Zheng, Tihua Zhang, Zhaoqiang Li, Sheng Li Liu, Shuqing Zheng, Qing Yin PLoS One Research Article 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. Public Library of Science 2013-11-12 /pmc/articles/PMC3827170/ /pubmed/24265785 http://dx.doi.org/10.1371/journal.pone.0079791 Text en © 2013 Sheykholeslami et al http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Sheykholeslami, Kianoush
Thimmappa, Vikrum
Nava, Casey
Bai, Xiaohui
Yu, Heping
Zheng, Tihua
Zhang, Zhaoqiang
Li, Sheng Li
Liu, Shuqing
Zheng, Qing Yin
A New Mutation of the Atoh1 Gene in Mice with Normal Life Span Allows Analysis of Inner Ear and Cerebellar Phenotype in Aging
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
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3827170/
https://www.ncbi.nlm.nih.gov/pubmed/24265785
http://dx.doi.org/10.1371/journal.pone.0079791
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