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Papers In Press, published online ahead of print June 13, 2002
Arthritis & Rheumatism Branch, NIAMS, National Institutes of Health, Bethesda, MD 20892-1820
Corresponding Author: plotzp{at}mail.nih.gov
Acid a-glucosidase (GAA) is a lysosomal enzyme that degrades glycogen. A deficiency of GAA is responsible for a recessively inherited myopathy and cardiomyopathy, glycogenosis type II (GSD II). Previously, we have identified an intronic repressor element in the GAA gene and demonstrated that Hes-1, a bHLH factor, binds to a C class E box within the element and functions as a transcriptional repressor in Hep G2 cells. Hes-1 is a well-studied downstream target gene in the Notch signaling pathway. In this study, over-expression and depletion of NICD (Notch-1 intracellular domain) strategies were used to investigate whether expression of the GAA gene is under the control of Notch-1/Hes-1 signaling. In co-transfection experiments, Hes-1, up-regulated by over-expressed NICD, enhanced the repressive effect of the DNA element with wild type Hes-1 binding sites, but not with mutant Hes-1 binding sites. Conversely, depletion of Notch-1 with phosphorothioated antisense oligonucleotides, corresponding to the fourth ankyrin repeat within NICD, led to reduced Hes-1. Constitutively over-expressed Hes-1 and Notch-1 repressed GAA gene expression. Therefore, our data establish that the human GAA gene, encoding a lysosomal enzyme, is a downstream target of the Notch-1/Hes-1 signaling pathway.
J. Biol. Chem, 10.1074/jbc.M204721200
Submitted on May 14, 2002
Revised on June 12, 2002
Accepted on June 13, 2002
The human acid alpha-glucosidase gene is a novel target of the Notch-1/Hes-1 signaling pathway
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