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

Merla, Giuseppe; Howald, Cedric; Antonarakis, Stylianos; Reymond, Alexandre

doi:10.1093/hmg/ddh163

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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
Authors		Merla, Giuseppe Howald, Cedric Antonarakis, Stylianos Reymond, Alexandre
Published in		Human Molecular Genetics. 2004, vol. 13, no. 14, p. 1505-1514
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		Animals — Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics/ 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/ metabolism — Transcriptional Activation — Two-Hybrid System Techniques — Williams Syndrome/ genetics
Identifiers		DOI: 10.1093/hmg/ddh163 PMID: 15163635
Full text		Article - Limited access to UNIGE Other version: http://hmg.oxfordjournals.org/cgi/reprint/13/14/1505.pdf
Structures		Faculté de médecine / Section de médecine clinique / Département de médecine
Citation (ISO format)		MERLA, Giuseppe et al. The subcellular localization of the ChoRE-binding protein, encoded by the Williams-Beuren syndrome critical region gene 14, is regulated by 14-3-3. In: Human Molecular Genetics, 2004, vol. 13, n° 14, p. 1505-1514. doi: 10.1093/hmg/ddh163 https://archive-ouverte.unige.ch/unige:8896