Gene regulation of Wiskott-Aldrich syndrome protein and the human homolog of the Drosophila Su(var)3-9: WASP and SUV39H1, two adjacent genes at Xp11.23

The region Xp11.23 is a gene-rich, light giemsa-staining segment on the sho rt arm of the X chromosome. In this study, we have characterized the transc riptional regulatory elements in this interval for two adjacent genes: SUV3 9H1, a regulator of chromatin organization, and the Wiskott-Aldrich syndrom e protein (WASP). The WASP gene exhibits two alternate promoters, both of w hich demonstrate transcription factor binding elements specific to blood ce ll lineages. Reporter gene expression analyses indicate that both WASP prom oters show high levels of expression in different hematopoietic cell lines. The human homolog of the Drosophila Su(var)3-9 gene was identified by sequ ence analysis of the region downstream from WASP. SUV39H1 is ubiquitously e xpressed, and the promoter sequence consists mostly of general transcriptio n factors. The presence of putative binding sites for GAGA and Adfl transcr iption factors may indicate a cross regulatory mechanism with other chromat in regulators. (C) 2000 Elsevier Science B.V. All rights reserved.