Document Detail


COUP-TFI controls Notch regulation of hair cell and support cell differentiation.
MedLine Citation:
PMID:  16914494     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The orphan nuclear receptor COUP-TFI (Nr2f1) regulates many aspects of mammalian development, but little is known about its role in cochlear hair cell and Deiter's support cell development. The COUP-TFI knockout (COUP-TFI(-/-)) has a significant increase in hair cell (HC) number in the mid-to-apical turns. The total number of hair cells is not increased over wild type, perhaps because of displaced hair cells and a shortened cochlear duct. This implicates a defect of convergent-extension in the COUP-TFI(-/-) duct. In addition, excess proliferation in the COUP-TFI(-/-) sensory epithelium indicates that the origin of the extra HCs in the apex is complex. Because loss-of-function studies of Notch signaling components have similar phenotypes, we investigated Notch regulation of hair cell differentiation in COUP-TFI(-/-) mice and confirmed misregulation of Notch signaling components, including Jag1, Hes5 and in a manner consistent with reduced Notch signaling, and correlated with increases in hair cell and support cell differentiation. The disruption of Notch signaling by a gamma-secretase inhibitor in an in vitro organ culture system of wild-type cochleae resulted in a reduction in expression of the Notch target gene Hes5 and an increase in hair cell differentiation. Importantly, inhibition of Notch activity resulted in a greater increase in hair cell differentiation in COUP-TFI(-/-) cochlear cultures than in wild-type cultures, suggesting a hypersensitivity to Notch inactivation in COUP-TFI(-/-) cochlea, particularly at the apical turn. Thus, we present evidence that reduced Notch signaling contributes to increases in hair cell and support cell differentiation in COUP-TFI(-/-) mice, and suggest that COUP-TFI is required for Notch regulation of hair cell and support cell differentiation.
Authors:
Louisa S Tang; Heather M Alger; Fred A Pereira
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural     Date:  2006-08-16
Journal Detail:
Title:  Development (Cambridge, England)     Volume:  133     ISSN:  0950-1991     ISO Abbreviation:  Development     Publication Date:  2006 Sep 
Date Detail:
Created Date:  2006-08-28     Completed Date:  2007-01-04     Revised Date:  2013-05-14    
Medline Journal Info:
Nlm Unique ID:  8701744     Medline TA:  Development     Country:  England    
Other Details:
Languages:  eng     Pagination:  3683-93     Citation Subset:  IM    
Affiliation:
Huffington Center on Aging, Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA.
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MeSH Terms
Descriptor/Qualifier:
Amyloid Precursor Protein Secretases / genetics,  metabolism
Animals
Basic Helix-Loop-Helix Transcription Factors / genetics,  metabolism
Blotting, Western
COUP Transcription Factor I / genetics,  metabolism,  physiology*
Cell Differentiation / drug effects,  genetics,  physiology*
Cell Proliferation / drug effects
Cells, Cultured
Cochlea / cytology,  embryology,  metabolism
Dose-Response Relationship, Drug
Fluorescent Antibody Technique
Gene Expression Regulation, Developmental / drug effects
Glycosyltransferases / genetics,  metabolism
Hair Cells, Auditory / cytology*,  drug effects,  metabolism
In Situ Hybridization
Mice
Mice, Knockout
Models, Biological
Organ of Corti / cytology,  embryology,  metabolism
Polymerase Chain Reaction / methods
Receptors, Notch / metabolism,  physiology*
Repressor Proteins / genetics,  metabolism
Signal Transduction / drug effects,  physiology
Triglycerides / pharmacology
gamma-Aminobutyric Acid / analogs & derivatives,  pharmacology
Grant Support
ID/Acronym/Agency:
DC04585/DC/NIDCD NIH HHS; R01 DC004585/DC/NIDCD NIH HHS; R55 DC004585/DC/NIDCD NIH HHS
Chemical
Reg. No./Substance:
0/Basic Helix-Loop-Helix Transcription Factors; 0/COUP Transcription Factor I; 0/Hes5 protein, mouse; 0/Receptors, Notch; 0/Repressor Proteins; 0/Triglycerides; 56-12-2/gamma-Aminobutyric Acid; 93349-26-9/1,2-dilinolenoyl-3-(4-aminobutyryl)propane-1,2,3-triol; EC 2.4.-/Glycosyltransferases; EC 2.4.1.-/Lfng protein, mouse; EC 3.4.-/Amyloid Precursor Protein Secretases

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