Ttv (EXT1). Impaired HS biosynthesis. Wing imaginal disc: defective Hh, Wg and Dpp distribution and signalling activity. Long range activity of Hh completely ...
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In format provided by Alman et al. (JULY 2010)
Supplementary Information S2 | Model organisms to study the formation of cartilaginous tumours with respect to EXT and growth plate signalling General phenotype Drosophila melanogaster (fruitfly) Ttv (EXT1) Impaired HS biosynthesis Wing imaginal disc: defective Hh, Wg and Dpp distribution and signalling activity Long range activity of Hh completely abolished Adult wing narrower in anterior/posterior orientation Mutant embryos die at pupal stage Sotv (EXT2) Impaired HS biosynthesis Wing imaginal disc: defective Hh, Wg and Dpp distribution and signalling activity Adult wing not as affected as ttv and botv mutants Botv (EXTL3) Impaired HS biosynthesis Wing imaginal disc: defective Hh, Wg and Dpp distribution and signalling activity Adult wing narrower in anterior/posterior orientation Danio rerio (zebrafish) Dackel (EXT2) All arches short and thick and strongly reduced, disorganized cartilage stacking No pectoral fins; tail often curls up Jaw not extended Impaired HS synthesis Boxer (EXTL3) All arches short and thick and strongly reduced No pectoral fins; tail often curls up Jaw not extended Impaired HS synthesis Caenorhabditis elegans (worm) Rib-1 (EXT1) Embryonic lethality Short, thick isthmus Impaired HS synthesis Rib-2 (EXT2) Egg laying defects Increased body width Reduced activity in movement Impaired HS synthesis
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1. Bellaiche, Y., The, I., & Perrimon, N. Tout-velu is a drosophila homologue of the putative tumour suppressor EXT1 and is needed for Hh diffusion. Nature 394, 8588 (1998). 2. The, I., Bellaiche, Y., & Perrimon, N. Hedgehog movement is regulated through tout velu -dependant synthesis of a heparan sulfate proteoglycan. Mol Cell 4(4), 633-639 (1999). 3. Han, C., et al. Distinct and collaborative roles of Drosophila EXT family proteins in morphogen signalling and gradient formation. Development 131(7), 1563-1575 (2004). 4. Takei, Y., Ozawa, Y., Sato, M., Watanabe, A., & Tabata, T. Three Drosophila EXT genes shape morphogen gradients through synthesis of heparan sulfate proteoglycans. Development 131(1), 73-82 (2004).
In format provided by Alman et al. (JULY 2010)
Hedgehog and Decapentaplegic signaling pathways. Development 131(9), 19271938 (2004). 6. Lee, J.S., et al. Axon sorting in the optic tract requires HSPG synthesis by ext2 (dackel) and extl3 (boxer). Neuron 44, 947-960 (2004). 7. Franks, D.M., Izumikawa, T., Kitagawa, H., Sugahara, K., & Okkema, P.G. C. elegans pharyngeal morphogenesis requires both de novo synthesis of pyrimidines and synthesis of heparan sulfate proteoglycans. Dev Biol 296, 409-420 (2006). 8. Morio, H., et al. EXT gene family member rib-2 is essential for embryonic development and heparan sulfate biosynthesis in Caenorhabditis elegans. Biochem Biophys Res Commun 301, 317323 (2003).
5. Bornemann, D.J., Duncan, J.E., Staatz, W., Selleck, S., & Warrior, R. Abrogation of heparan sulfate synthesis in Drosophila disrupts the Wingless,
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