Specific interaction between the XNP/ATR-X gene product and the SET domain of the human EZH2 protein.

Abstract	Mutations in the XNP gene result in different inherited disorders, including the ATR-X syndrome which is characterized by mental retardation (MR) associated with alpha-thalaessemia. Amino acid sequence analysis revealed that the XNP protein is a new member of the SNF2-like family, which comprises numerous members involved in a broad range of biological functions: transcriptional regulation, DNA repair and chromosome segregation. Since experiments on fibroblasts from ATR-X patients have provided no evidence for either a DNA repair defect or abnormal chromosome breakage or segregation, it seems more likely that the XNP protein is somehow involved in regulation of gene expression. Recent genetic and biochemical studies have led to the emerging concept that SNF2-like proteins are components of a large protein complex which may exert its functions by modulating chromatin structure. To investigate whether XNP could mediate the activity of gene-specific activators through chromatin remodelling, we performed a yeast two-hybrid analysis using XNP and several human heterochromatin-associated proteins. We found a specific interaction between the XNP and the EZH2 proteins. In light of these observations, we discuss how the XNP protein may regulate gene transcription at the chromatin level.
Authors	C Cardoso, S Timsit, L Villard, M Khrestchatisky, M Fontès, L Colleaux
Journal	Human molecular genetics (Hum Mol Genet) Vol. 7 Issue 4 Pg. 679-84 (Apr 1998) ISSN: 0964-6906 [Print] England
PMID	9499421 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	BMI1 protein, human Bmi1 protein, mouse Bmi1 protein, rat DNA-Binding Proteins Drosophila Proteins Nuclear Proteins PCGF2 protein, human Polycomb-Group Proteins Proto-Oncogene Proteins Recombinant Fusion Proteins Repressor Proteins Transcription Factors E(z) protein, Drosophila Polycomb Repressive Complex 2 Polycomb Repressive Complex 1 beta-Galactosidase DNA Helicases ATRX protein, human Atrx protein, mouse X-linked Nuclear Protein
Topics	Animals DNA Helicases (genetics, metabolism) DNA-Binding Proteins (genetics) Drosophila Proteins Gene Expression Regulation Humans In Situ Hybridization Nuclear Proteins (chemistry, genetics, metabolism) Polycomb Repressive Complex 1 Polycomb Repressive Complex 2 Polycomb-Group Proteins Proto-Oncogene Proteins (genetics) Rats Recombinant Fusion Proteins (genetics, metabolism) Repressor Proteins (genetics, metabolism) Saccharomyces cerevisiae (genetics) Tissue Distribution Transcription Factors (genetics, metabolism) X-linked Nuclear Protein Zinc Fingers (genetics) beta-Galactosidase (metabolism)

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