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

Full 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.

Author information

Author/s: Maricich, Stephen M (SM); Xia, Anping (A); Mathes, Erin L (EL); Wang, Vincent Y (VY); Oghalai, John S (JS); Fritzsch, Bernd (B); Zoghbi, Huda Y (HY);

Affiliation: Department of Pediatrics and Neurosciences, Case Western Reserve University, Cleveland, Ohio 44106, USA. smm18(-atsign-)case.edu

Grants: HD024064 (Agency:NICHD NIH HHS) ; K08 DC006671-04 (Agency:NIDCD NIH HHS) ; K08 NS053419-04 (Agency:NINDS NIH HHS) ; K08DC006671 (Agency:NIDCD NIH HHS) ; K08NS053419 (Agency:NINDS NIH HHS) ; P30 HD024064-21 (Agency:NICHD NIH HHS) ; R01 DC005590-05 (Agency:NIDCD NIH HHS) ; R01DC005590 (Agency:NIDCD NIH HHS) ; (Agency:Howard Hughes Medical Institute)

Journal and publication information

Publication Type: Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

Journal: The Journal of neuroscience : the official journal of the Society for Neuroscience (J Neurosci), published in United States. (Language: eng)

Reference: 2009-Sep; vol 29 (issue 36) : pp 11123-33

Dates: Created 2009/09/10; Completed 2009/09/28;

PMID: 19741118, status: MEDLINE (last retrieval date: 9/28/2009, IMS Date: )

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

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