Human Molecular Genetics, Vol 7, 679-684, Copyright © 1998 by Oxford University Press
C Cardoso, S Timsit, L Villard, M Khrestchatisky, M Fontes and L Colleaux
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.
ARTICLES
Specific interaction between the XNP/ATR-X gene product and the SET domain of the human EZH2 protein
Unit INSERM U406, Genetique Medicale et Developpement, Faculte de Medecine de la Timone, 27 Boulevard Jean Moulin, 13385 Marseille cedex 05, France.
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