Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei

Stephen M Maricich; Anping Xia; Erin L Mathes; Vincent Y Wang; John S Oghalai; Bernd Fritzsch; Huda Y Zoghbi

doi:10.1523/JNEUROSCI.2232-09.2009

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Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei

Journal article

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Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei

Stephen M Maricich, Anping Xia, Erin L Mathes, Vincent Y Wang, John S Oghalai, Bernd Fritzsch and Huda Y Zoghbi

The Journal of neuroscience, Vol.29(36), pp.11123-11133

09/09/2009

DOI: 10.1523/JNEUROSCI.2232-09.2009

PMCID: PMC2743121

PMID: 19741118

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Abstract

Atoh1 is a basic helix-loop-helix transcription factor necessary for the specification of inner ear hair cells and central auditory system neurons derived from the rhombic lip. We used the Cre-loxP system and two Cre-driver lines (Egr2(Cre) and Hoxb1(Cre)) to delete Atoh1 from different regions of the cochlear nucleus (CN) and accessory auditory nuclei (AAN). Adult Atoh1-conditional knock-out mice (Atoh1(CKO)) are behaviorally deaf, have diminished auditory brainstem evoked responses, and have disrupted CN and AAN morphology and connectivity. In addition, Egr2; Atoh1(CKO) mice lose spiral ganglion neurons in the cochlea and AAN neurons during the first 3 d of life, revealing a novel critical period in the development of these neurons. These new mouse models of predominantly central deafness illuminate the importance of the CN for support of a subset of peripheral and central auditory neurons.

Animals, Newborn

Neurons - pathology

Basic Helix-Loop-Helix Transcription Factors - physiology

Basic Helix-Loop-Helix Transcription Factors - genetics

Cochlear Nucleus - physiology

Hearing - genetics

Spiral Ganglion - physiology

Mice, Inbred C57BL

Mice, Transgenic

Cochlear Nucleus - pathology

Brain Stem - physiology

Mice, Knockout

Acoustic Stimulation - methods

Pregnancy

Animals

Brain Stem - pathology

Neurons - physiology

Spiral Ganglion - pathology

Female

Hearing - physiology

Mice

Mice, Congenic

Basic Helix-Loop-Helix Transcription Factors - deficiency

Cell Survival - physiology

Details

Title: Subtitle: Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei
Creators: Stephen M Maricich - Department of Pediatrics and Neurosciences, Case Western Reserve University, Cleveland, Ohio 44106, USA. smm18@case.edu
Anping Xia
Erin L Mathes
Vincent Y Wang
John S Oghalai
Bernd Fritzsch
Huda Y Zoghbi
Resource Type: Journal article
Publication Details: The Journal of neuroscience, Vol.29(36), pp.11123-11133
DOI: 10.1523/JNEUROSCI.2232-09.2009
PMID: 19741118
PMCID: PMC2743121
NLM abbreviation: J Neurosci
ISSN: 0270-6474
eISSN: 1529-2401
Publisher: United States
Grant note: Howard Hughes Medical Institute K08 NS053419-04 / NINDS NIH HHS P30 HD024064-21 / NICHD NIH HHS P30 HD024064 / NICHD NIH HHS HD024064 / NICHD NIH HHS R01 DC005590-05 / NIDCD NIH HHS K08 NS053419 / NINDS NIH HHS K08 DC006671-04 / NIDCD NIH HHS K08 DC006671 / NIDCD NIH HHS R01 DC005590 / NIDCD NIH HHS K08DC006671 / NIDCD NIH HHS K08NS053419 / NINDS NIH HHS R01DC005590 / NIDCD NIH HHS
Language: English
Date published: 09/09/2009
Academic Unit: Iowa Neuroscience Institute; Biology; Craniofacial Anomalies Research Center
Record Identifier: 9984070678702771

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