Document Detail

The rod photoreceptor-specific nuclear receptor Nr2e3 represses transcription of multiple cone-specific genes.
MedLine Citation:
PMID:  15634773     Owner:  NLM     Status:  MEDLINE    
This study addresses one genetic regulatory mechanism that establishes the distinct identities of rod and cone photoreceptors. Previous work has shown that mutations in either humans or mice in the gene coding for photoreceptor-specific nuclear receptor Nr2e3 cause a progressive retinal degeneration characterized by increased numbers of short-wave cones. In the present work, we have examined the cellular and developmental pattern of Nr2e3 protein localization in mammals and fish, identified an optimal Nr2e3 DNA-binding site using cycles of binding to recombinant Nr2e3, characterized the transcriptional activity of wild type and one of the disease-associated point mutations in Nr2e3 in transfected cells, and characterized the transcriptional defects in the naturally occurring Nr2e3 mutant (rd7) mouse. These experiments indicate that in the mature vertebrate retina Nr2e3 is expressed exclusively in rods, that expression of Nr2e3 is one of the earliest events in the pathway of rod-specific photoreceptor development, and that Nr2e3 functions, either directly or indirectly, as a repressor of cone-specific genes in rod photoreceptor cells.
Jichao Chen; Amir Rattner; Jeremy Nathans
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of neuroscience : the official journal of the Society for Neuroscience     Volume:  25     ISSN:  1529-2401     ISO Abbreviation:  J. Neurosci.     Publication Date:  2005 Jan 
Date Detail:
Created Date:  2005-01-06     Completed Date:  2005-07-20     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8102140     Medline TA:  J Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  118-29     Citation Subset:  IM    
Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
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MeSH Terms
Amino Acid Sequence
Cells, Cultured
Conserved Sequence
DNA-Binding Proteins / physiology
Eye Proteins / biosynthesis,  physiology*
Mice, Mutant Strains
Molecular Sequence Data
Nucleic Acid Hybridization
Oligonucleotide Array Sequence Analysis
Orphan Nuclear Receptors
Point Mutation
Receptors, Cytoplasmic and Nuclear / biosynthesis,  genetics,  physiology*
Repressor Proteins / biosynthesis,  physiology*
Retinal Cone Photoreceptor Cells / metabolism*
Retinal Rod Photoreceptor Cells / metabolism,  physiology*
Transcription Factors / biosynthesis,  genetics,  physiology*
Transducin / analysis
Reg. No./Substance:
0/DNA-Binding Proteins; 0/Eye Proteins; 0/NR2E3 protein, human; 0/Nr2e3 protein, mouse; 0/Orphan Nuclear Receptors; 0/Receptors, Cytoplasmic and Nuclear; 0/Repressor Proteins; 0/Transcription Factors; EC 3.6.1.-/Transducin

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