The subcellular localization of the ChoRE-binding protein, encoded by the Williams-Beuren syndrome critical region gene 14, is regulated by 14-3-3.

Details

Serval ID
serval:BIB_E12EA2517C19
Type
Article: article from journal or magazin.
Collection
Publications
Title
The subcellular localization of the ChoRE-binding protein, encoded by the Williams-Beuren syndrome critical region gene 14, is regulated by 14-3-3.
Journal
Human Molecular Genetics
Author(s)
Merla G., Howald C., Antonarakis S.E., Reymond A.
ISSN
0964-6906[print], 0964-6906[linking]
Publication state
Published
Issued date
2004
Peer-reviewed
Oui
Volume
13
Number
14
Pages
1505-1514
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
The Williams-Beuren syndrome (WBS) is a contiguous gene syndrome caused by chromosomal rearrangements at chromosome band 7q11.23. Several endocrine phenotypes, in particular impaired glucose tolerance and silent diabetes, have been described for this clinically complex disorder. The WBSCR14 gene, one of the genes mapping to the WBS critical region, encodes a member of the basic-helix-loop-helix leucine zipper family of transcription factors, which dimerizes with the Max-like protein, Mlx. This heterodimeric complex binds and activates, in a glucose-dependent manner, carbohydrate response element (ChoRE) motifs in the promoter of lipogenic enzymes. We identified five novel WBSCR14-interacting proteins, four 14-3-3 isotypes and NIF3L1, which form a single polypeptide complex in mammalian cells. Phosphatase treatment abrogates the association between WBSCR14 and 14-3-3, as shown previously for multiple 14-3-3 interactors. WBSCR14 is exported actively from the nucleus through a CRM1-dependent mechanism. This translocation is contingent upon the ability to bind 14-3-3. Through this mechanism the 14-3-3 isotypes directly affect the WBSCR14:Mlx complexes, which activate the transcription of lipogenic genes.
Keywords
14-3-3 Proteins/genetics, 14-3-3 Proteins/metabolism, Animals, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/metabolism, Cells, Cultured, Cercopithecus aethiops, Chromosome Mapping, Chromosomes, Human, Pair 7, Helix-Loop-Helix Motifs, Humans, Promoter Regions, Genetic, Proteins/metabolism, Response Elements, Transcription Factors/genetics, Transcription Factors/metabolism, Transcriptional Activation, Two-Hybrid System Techniques, Williams Syndrome/genetics
Pubmed
Web of science
Open Access
Yes
Create date
24/01/2008 16:52
Last modification date
20/08/2019 17:05
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