Functional conservation of the human EXT1 tumor suppressor gene and its Drosophila homolog toutvelu |
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| Authors: | Ujjaini Dasgupta Bharat L. Dixit Melissa Rusch Scott Selleck Inge The |
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| Affiliation: | (1) Program in Gene Function and Expression, University of Massachusetts Medical School, 364 Plantation Street, Worcester, MA 01605, USA;(2) Department of Pediatrics, The Developmental Biology Center, University of Minnesota, 6-160 Jackson Hall, 321 Church Street SE, Minneapolis, MN 55455, USA;(3) Department of Genetics, Cell Biology and Development, The Developmental Biology Center, University of Minnesota, 6-160 Jackson Hall, 321 Church Street SE, Minneapolis, MN 55455, USA;(4) Present address: Division of Developmental Biology, Universiteit Utrecht, Padualaan 8, 3584 CH Utrecht, The Netherlands |
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| Abstract: | Heparan sulfate proteoglycans play a vital role in signaling of various growth factors in both Drosophila and vertebrates. In Drosophila, mutations in the tout velu (ttv) gene, a homolog of the mammalian EXT1 tumor suppressor gene, leads to abrogation of glycosaminoglycan (GAG) biosynthesis. This impairs distribution and signaling activities of various morphogens such as Hedgehog (Hh), Wingless (Wg), and Decapentaplegic (Dpp). Mutations in members of the exostosin (EXT) gene family lead to hereditary multiple exostosis in humans leading to bone outgrowths and tumors. In this study, we provide genetic and biochemical evidence that the human EXT1 (hEXT1) gene is conserved through species and can functionally complement the ttv mutation in Drosophila. The hEXT1 gene was able to rescue a ttv null mutant to adulthood and restore GAG biosynthesis. |
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| Keywords: | Drosophila HSPGs EXT Conservation Evolution |
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