| The rod photoreceptor-specific nuclear receptor Nr2e3 represses transcription of multiple cone-specific genes. | |
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MedLine Citation:
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PMID: 15634773 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Jichao Chen; Amir Rattner; Jeremy Nathans |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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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:
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Created Date: 2005-01-06 Completed Date: 2005-07-20 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 8102140 Medline TA: J Neurosci Country: United States |
Other Details:
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Languages: eng Pagination: 118-29 Citation Subset: IM |
Affiliation:
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Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Amino Acid Sequence Animals Cells, Cultured Conserved Sequence DNA-Binding Proteins / physiology Eye Proteins / biosynthesis, physiology* Humans Macaca Mice 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 Transfection Zebrafish |
| Chemical | |
Reg. No./Substance:
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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 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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