TY - JOUR
T1 - Functional conservation of the human EXT1 tumor suppressor gene and its Drosophila homolog tout velu
AU - Dasgupta, Ujjaini
AU - Dixit, Bharat L.
AU - Rusch, Melissa
AU - Selleck, Scott
AU - The, Inge
N1 - Funding Information:
Acknowledgments We thank Peter S. Bak and Hau Hung for generating the hEXT1-GFP lines. We thank Frank Tufaro, Pernille Rorth, Xinhua Lin, and John Sisson for sending us reagents. We also thank the UMass Medical School Drosophila community for interesting discussions and suggestions. This work was funded by grants from the National Institutes of Health (GM066220 to IT and GM54832 to SBS).
PY - 2007/8
Y1 - 2007/8
N2 - 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.
AB - 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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U2 - 10.1007/s00427-007-0163-2
DO - 10.1007/s00427-007-0163-2
M3 - Article
C2 - 17610078
AN - SCOPUS:38449095149
SN - 0949-944X
VL - 217
SP - 555
EP - 561
JO - Development Genes and Evolution
JF - Development Genes and Evolution
IS - 8
ER -