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Papers In Press, published online ahead of print October 18, 2000
J. Biol. Chem, 10.1074/jbc.M005959200
Submitted on July 6, 2000
Revised on October 13, 2000
Accepted on October 17, 2000
-glucosidase gene: Identification of a repressor element and its transcription factors - Hes-1 and YY1
Arthritis & Rheumatism Branch, NIAMS, National Institutes of Health, Bethesda, MD 20892-1820
Corresponding Author: plotzp{at}mail.nih.gov
Acid a-glucosidase, the product of a housekeeping gene, is a lysosomal enzyme that degrades glycogen. A deficiency of this enzyme is responsible for a recessively inherited myopathy and cardiomyopathy, glycogenosis type II. We have previously demonstrated that the human acid a-glucosidase gene expression is regulated by a silencer within intron 1, which is located in the 5' untranslated region. In this study, we have used deletion analysis, electrophoretic mobility shift assay and footprint analysis to further localize the silencer to a 25bp element. The repressive effect on the TK promoter was about 50% in both orientations in expression plasmid, and two transcriptional factors were identified with antibodies binding specifically to the element. Mutagenesis and functional analyses of the element demonstrated that the mammalian homologue 1 of Drosophila hairy and Enhancer of split (Hes-1) binding to an E box (CACGCG) and global transcription factor-YY1 binding to its core site, function as a transcriptional repressor. Furthermore, over-expression of Hes-1 significantly enhanced the repressive effect of the silencer element. The data should be helpful in understanding the expression and regulation of the human acid a-glucosidase gene as well as other lysosomal genes.
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