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Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems
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Zeitschriftentitel: | Genes & Development |
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Personen und Körperschaften: | , , , , , , , , , |
In: | Genes & Development, 14, 2000, 22, S. 2839-2854 |
Format: | E-Article |
Sprache: | Englisch |
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Cold Spring Harbor Laboratory
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author_facet |
Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer |
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author |
Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer |
spellingShingle |
Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer Genes & Development Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems Developmental Biology Genetics |
author_sort |
liu, min |
spelling |
Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer 0890-9369 1549-5477 Cold Spring Harbor Laboratory Developmental Biology Genetics http://dx.doi.org/10.1101/gad.840500 <jats:p><jats:italic>BETA2/NeuroD1</jats:italic> is a bHLH transcription factor that is expressed during development in the mammalian pancreas and in many locations in the central and peripheral nervous systems. During inner ear ontogenesis, it is present in both sensory ganglion neurons and sensory epithelia. Although studies have shown that<jats:italic>BETA2/NeuroD1</jats:italic> is important in the development of the hippocampal dentate gyrus and the cerebellum, its functions in the peripheral nervous system and in particular in the inner ear are unclear. Mice carrying a <jats:italic>BETA2/NeuroD1</jats:italic> null mutation exhibit behavioral abnormalities suggestive of an inner ear defect, including lack of responsiveness to sound, hyperactivity, head tilting, and circling. Here we show that these defects can be explained by a severe reduction of sensory neurons in the cochlear-vestibular ganglion (CVG). A developmental study of CVG formation in the null demonstrates that BETA2/NeuroD1 does not play a primary role in the proliferation of neuroblast precursors or in their decision to become neuroblasts. Instead, the reduction in CVG neuron number is caused by a combination both of delayed or defective delamination of CVG neuroblast precursors from the otic vesicle epithelium and of enhanced apoptosis both in the otic epithelium and among those neurons that do delaminate to form the CVG. There are also defects in differentiation and patterning of the cochlear duct and sensory epithelium and loss of the dorsal cochlear nucleus. <jats:italic>BETA2/NeuroD1</jats:italic> is, thus, the first gene to be shown to regulate neuronal and sensory cell development in both the cochlear and vestibular systems.</jats:p> Essential role of <i>BETA2/NeuroD1</i> in development of the vestibular and auditory systems Genes & Development |
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10.1101/gad.840500 |
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Cold Spring Harbor Laboratory, 2000 |
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title |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_unstemmed |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_full |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_fullStr |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_full_unstemmed |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_short |
Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_sort |
essential role of <i>beta2/neurod1</i> in development of the vestibular and auditory systems |
topic |
Developmental Biology Genetics |
url |
http://dx.doi.org/10.1101/gad.840500 |
publishDate |
2000 |
physical |
2839-2854 |
description |
<jats:p><jats:italic>BETA2/NeuroD1</jats:italic> is a bHLH transcription factor that is expressed during development in the mammalian pancreas and in many locations in the central and peripheral nervous systems. During inner ear ontogenesis, it is present in both sensory ganglion neurons and sensory epithelia. Although studies have shown that<jats:italic>BETA2/NeuroD1</jats:italic> is important in the development of the hippocampal dentate gyrus and the cerebellum, its functions in the peripheral nervous system and in particular in the inner ear are unclear. Mice carrying a <jats:italic>BETA2/NeuroD1</jats:italic> null mutation exhibit behavioral abnormalities suggestive of an inner ear defect, including lack of responsiveness to sound, hyperactivity, head tilting, and circling. Here we show that these defects can be explained by a severe reduction of sensory neurons in the cochlear-vestibular ganglion (CVG). A developmental study of CVG formation in the null demonstrates that BETA2/NeuroD1 does not play a primary role in the proliferation of neuroblast precursors or in their decision to become neuroblasts. Instead, the reduction in CVG neuron number is caused by a combination both of delayed or defective delamination of CVG neuroblast precursors from the otic vesicle epithelium and of enhanced apoptosis both in the otic epithelium and among those neurons that do delaminate to form the CVG. There are also defects in differentiation and patterning of the cochlear duct and sensory epithelium and loss of the dorsal cochlear nucleus. <jats:italic>BETA2/NeuroD1</jats:italic> is, thus, the first gene to be shown to regulate neuronal and sensory cell development in both the cochlear and vestibular systems.</jats:p> |
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author | Liu, Min, Pereira, Fred A., Price, Steven D., Chu, Mei-jin, Shope, Cindy, Himes, Donna, Eatock, Ruth Anne, Brownell, William E., Lysakowski, Anna, Tsai, Ming-Jer |
author_facet | Liu, Min, Pereira, Fred A., Price, Steven D., Chu, Mei-jin, Shope, Cindy, Himes, Donna, Eatock, Ruth Anne, Brownell, William E., Lysakowski, Anna, Tsai, Ming-Jer, Liu, Min, Pereira, Fred A., Price, Steven D., Chu, Mei-jin, Shope, Cindy, Himes, Donna, Eatock, Ruth Anne, Brownell, William E., Lysakowski, Anna, Tsai, Ming-Jer |
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description | <jats:p><jats:italic>BETA2/NeuroD1</jats:italic> is a bHLH transcription factor that is expressed during development in the mammalian pancreas and in many locations in the central and peripheral nervous systems. During inner ear ontogenesis, it is present in both sensory ganglion neurons and sensory epithelia. Although studies have shown that<jats:italic>BETA2/NeuroD1</jats:italic> is important in the development of the hippocampal dentate gyrus and the cerebellum, its functions in the peripheral nervous system and in particular in the inner ear are unclear. Mice carrying a <jats:italic>BETA2/NeuroD1</jats:italic> null mutation exhibit behavioral abnormalities suggestive of an inner ear defect, including lack of responsiveness to sound, hyperactivity, head tilting, and circling. Here we show that these defects can be explained by a severe reduction of sensory neurons in the cochlear-vestibular ganglion (CVG). A developmental study of CVG formation in the null demonstrates that BETA2/NeuroD1 does not play a primary role in the proliferation of neuroblast precursors or in their decision to become neuroblasts. Instead, the reduction in CVG neuron number is caused by a combination both of delayed or defective delamination of CVG neuroblast precursors from the otic vesicle epithelium and of enhanced apoptosis both in the otic epithelium and among those neurons that do delaminate to form the CVG. There are also defects in differentiation and patterning of the cochlear duct and sensory epithelium and loss of the dorsal cochlear nucleus. <jats:italic>BETA2/NeuroD1</jats:italic> is, thus, the first gene to be shown to regulate neuronal and sensory cell development in both the cochlear and vestibular systems.</jats:p> |
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spelling | Liu, Min Pereira, Fred A. Price, Steven D. Chu, Mei-jin Shope, Cindy Himes, Donna Eatock, Ruth Anne Brownell, William E. Lysakowski, Anna Tsai, Ming-Jer 0890-9369 1549-5477 Cold Spring Harbor Laboratory Developmental Biology Genetics http://dx.doi.org/10.1101/gad.840500 <jats:p><jats:italic>BETA2/NeuroD1</jats:italic> is a bHLH transcription factor that is expressed during development in the mammalian pancreas and in many locations in the central and peripheral nervous systems. During inner ear ontogenesis, it is present in both sensory ganglion neurons and sensory epithelia. Although studies have shown that<jats:italic>BETA2/NeuroD1</jats:italic> is important in the development of the hippocampal dentate gyrus and the cerebellum, its functions in the peripheral nervous system and in particular in the inner ear are unclear. Mice carrying a <jats:italic>BETA2/NeuroD1</jats:italic> null mutation exhibit behavioral abnormalities suggestive of an inner ear defect, including lack of responsiveness to sound, hyperactivity, head tilting, and circling. Here we show that these defects can be explained by a severe reduction of sensory neurons in the cochlear-vestibular ganglion (CVG). A developmental study of CVG formation in the null demonstrates that BETA2/NeuroD1 does not play a primary role in the proliferation of neuroblast precursors or in their decision to become neuroblasts. Instead, the reduction in CVG neuron number is caused by a combination both of delayed or defective delamination of CVG neuroblast precursors from the otic vesicle epithelium and of enhanced apoptosis both in the otic epithelium and among those neurons that do delaminate to form the CVG. There are also defects in differentiation and patterning of the cochlear duct and sensory epithelium and loss of the dorsal cochlear nucleus. <jats:italic>BETA2/NeuroD1</jats:italic> is, thus, the first gene to be shown to regulate neuronal and sensory cell development in both the cochlear and vestibular systems.</jats:p> Essential role of <i>BETA2/NeuroD1</i> in development of the vestibular and auditory systems Genes & Development |
spellingShingle | Liu, Min, Pereira, Fred A., Price, Steven D., Chu, Mei-jin, Shope, Cindy, Himes, Donna, Eatock, Ruth Anne, Brownell, William E., Lysakowski, Anna, Tsai, Ming-Jer, Genes & Development, Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems, Developmental Biology, Genetics |
title | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_full | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_fullStr | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_full_unstemmed | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_short | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
title_sort | essential role of <i>beta2/neurod1</i> in development of the vestibular and auditory systems |
title_unstemmed | Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems |
topic | Developmental Biology, Genetics |
url | http://dx.doi.org/10.1101/gad.840500 |