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Originally published In Press as doi:10.1074/jbc.M204721200 on June 13, 2002

J. Biol. Chem., Vol. 277, Issue 33, 29760-29764, August 16, 2002
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The Human Acid alpha -Glucosidase Gene Is a Novel Target of the Notch-1/Hes-1 Signaling Pathway*

Bo YanDagger , Nina Raben, and Paul Plotz§

From the Arthritis and Rheumatism Branch, NIAMS, National Institutes of Health, Bethesda, Maryland 20892-1820

Acid alpha -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. Previously, we identified an intronic repressor element in the GAA gene and demonstrated that Hes-1, a basic helix-loop-helix factor, binds to a C class E box within the element and functions as a transcriptional repressor in HepG2 cells. Hes-1 is a well studied downstream target gene in the Notch signaling pathway. In this study, over-expression and depletion of Notch-1 intracellular domain (NICD) 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.

* The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Dagger Current address: Dept. of Anatomy and Cell Biology, George Washington University, Washington, D. C. 20037.

§ To whom correspondence should be addressed: Clinical Center, Bldg. 10, Rm. 9N244, NIAMS, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892-1820. Tel.: 301-496-1474; Fax: 301-402-0012; E-mail: plotzp@mail.nih.gov.

Copyright © 2002 by The American Society for Biochemistry and Molecular Biology, Inc.
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