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39 results on '"Purgato, Stefania"'

3. Author Correction: Comparative and demographic analysis of orang-utan genomes

Author

Locke, Devin P., Hillier, LaDeana W., Warren, Wesley C., Worley, Kim C., Nazareth, Lynne V., Muzny, Donna M., Yang, Shiaw-Pyng, Wang, Zhengyuan, Chinwalla, Asif T., Minx, Pat, Mitreva, Makedonka, Cook, Lisa, Delehaunty, Kim D., Fronick, Catrina, Schmidt, Heather, Fulton, Lucinda A., Fulton, Robert S., Nelson, Joanne O., Magrini, Vincent, Pohl, Craig, Graves, Tina A., Markovic, Chris, Cree, Andy, Dinh, Huyen H., Hume, Jennifer, Kovar, Christie L., Fowler, Gerald R., Lunter, Gerton, Meader, Stephen, Heger, Andreas, Ponting, Chris P., Marques-Bonet, Tomas, Alkan, Can, Chen, Lin, Cheng, Ze, Kidd, Jeffrey M., Eichler, Evan E., White, Simon, Searle, Stephen, Vilella, Albert J., Chen, Yuan, Flicek, Paul, Ma, Jian, Raney, Brian, Suh, Bernard, Burhans, Richard, Herrero, Javier, Haussler, David, Faria, Rui, Fernando, Olga, Darré, Fleur, Farré, Domènec, Gazave, Elodie, Oliva, Meritxell, Navarro, Arcadi, Roberto, Roberta, Capozzi, Oronzo, Archidiacono, Nicoletta, Della Valle, Giuliano, Purgato, Stefania, Rocchi, Mariano, Konkel, Miriam K., Walker, Jerilyn A., Ullmer, Brygg, Batzer, Mark A., Smit, Arian F. A., Hubley, Robert, Casola, Claudio, Schrider, Daniel R., Hahn, Matthew W., Quesada, Victor, Puente, Xose S., Ordoñez, Gonzalo R., López-Otín, Carlos, Vinar, Tomas, Brejova, Brona, Ratan, Aakrosh, Harris, Robert S., Miller, Webb, Kosiol, Carolin, Lawson, Heather A., Taliwal, Vikas, Martins, André L., Siepel, Adam, RoyChoudhury, Arindam, Ma, Xin, Degenhardt, Jeremiah, Bustamante, Carlos D., Gutenkunst, Ryan N., Mailund, Thomas, Dutheil, Julien Y., Hobolth, Asger, Schierup, Mikkel H., Ryder, Oliver A., Yoshinaga, Yuko, de Jong, Pieter J., Weinstock, George M., Rogers, Jeffrey, Mardis, Elaine R., Gibbs, Richard A., and Wilson, Richard K.

Published
2022
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6. Supplementary Figure 5 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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7. Supplementary Figure 2 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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8. CCR Translation for This Article from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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9. Supplementary Figure 3 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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10. Supplementary Figure 6 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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11. Supplementary Tables 1-3 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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12. Supplementary Tables 6-7 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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13. Supplementary Figure 4 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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14. Supplementary Materials and Methods from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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15. Supplementary Figure 1 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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16. Supplementary Table 4 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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17. Supplementary Table 5 from Antitumor Activity of Sustained N-Myc Reduction in Rhabdomyosarcomas and Transcriptional Block by Antigene Therapy

Author

Tonelli, Roberto, primary, McIntyre, Alan, primary, Camerin, Consuelo, primary, Walters, Zoë S., primary, Di Leo, Korinne, primary, Selfe, Joanna, primary, Purgato, Stefania, primary, Missiaglia, Edoardo, primary, Tortori, Andrea, primary, Renshaw, Jane, primary, Astolfi, Annalisa, primary, Taylor, Kathryn R., primary, Serravalle, Salvatore, primary, Bishop, Ryan, primary, Nanni, Cristina, primary, Valentijn, Linda J., primary, Faccini, Andrea, primary, Leuschner, Ivo, primary, Formica, Serena, primary, Reis-Filho, Jorge S., primary, Ambrosini, Valentina, primary, Thway, Khin, primary, Franzoni, Monica, primary, Summersgill, Brenda, primary, Marchelli, Rosangela, primary, Hrelia, Patrizia, primary, Cantelli-Forti, Giorgio, primary, Fanti, Stefano, primary, Corradini, Roberto, primary, Pession, Andrea, primary, and Shipley, Janet, primary

Published
2023
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18. Supplementary Figures from MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., primary, Maritz, Michelle F., primary, Liu, Bing, primary, Kong, Cheng Fei, primary, Purgato, Stefania, primary, Yang, Chen, primary, Murray, Jayne, primary, Russell, Amanda J., primary, Flemming, Claudia L., primary, von Jonquieres, Georg, primary, Pickett, Hilda A., primary, London, Wendy B., primary, Haber, Michelle, primary, Gunaratne, Preethi H., primary, Norris, Murray D., primary, Perini, Giovanni, primary, Fletcher, Jamie I., primary, and MacKenzie, Karen L., primary

Published
2023
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19. Supplementary Figure Legends from MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., primary, Maritz, Michelle F., primary, Liu, Bing, primary, Kong, Cheng Fei, primary, Purgato, Stefania, primary, Yang, Chen, primary, Murray, Jayne, primary, Russell, Amanda J., primary, Flemming, Claudia L., primary, von Jonquieres, Georg, primary, Pickett, Hilda A., primary, London, Wendy B., primary, Haber, Michelle, primary, Gunaratne, Preethi H., primary, Norris, Murray D., primary, Perini, Giovanni, primary, Fletcher, Jamie I., primary, and MacKenzie, Karen L., primary

Published
2023
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20. Supplementary Methods from MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., primary, Maritz, Michelle F., primary, Liu, Bing, primary, Kong, Cheng Fei, primary, Purgato, Stefania, primary, Yang, Chen, primary, Murray, Jayne, primary, Russell, Amanda J., primary, Flemming, Claudia L., primary, von Jonquieres, Georg, primary, Pickett, Hilda A., primary, London, Wendy B., primary, Haber, Michelle, primary, Gunaratne, Preethi H., primary, Norris, Murray D., primary, Perini, Giovanni, primary, Fletcher, Jamie I., primary, and MacKenzie, Karen L., primary

Published
2023
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21. Supplementary Figure 6 from MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., primary, Maritz, Michelle F., primary, Liu, Bing, primary, Kong, Cheng Fei, primary, Purgato, Stefania, primary, Yang, Chen, primary, Murray, Jayne, primary, Russell, Amanda J., primary, Flemming, Claudia L., primary, von Jonquieres, Georg, primary, Pickett, Hilda A., primary, London, Wendy B., primary, Haber, Michelle, primary, Gunaratne, Preethi H., primary, Norris, Murray D., primary, Perini, Giovanni, primary, Fletcher, Jamie I., primary, and MacKenzie, Karen L., primary

Published
2023
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22. Supplementary Tables from MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., primary, Maritz, Michelle F., primary, Liu, Bing, primary, Kong, Cheng Fei, primary, Purgato, Stefania, primary, Yang, Chen, primary, Murray, Jayne, primary, Russell, Amanda J., primary, Flemming, Claudia L., primary, von Jonquieres, Georg, primary, Pickett, Hilda A., primary, London, Wendy B., primary, Haber, Michelle, primary, Gunaratne, Preethi H., primary, Norris, Murray D., primary, Perini, Giovanni, primary, Fletcher, Jamie I., primary, and MacKenzie, Karen L., primary

Published
2023
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24. Centromere sliding on a mammalian chromosome

Author

Purgato, Stefania, Belloni, Elisa, Piras, Francesca M., Zoli, Monica, Badiale, Claudia, Cerutti, Federico, Mazzagatti, Alice, Perini, Giovanni, Della Valle, Giuliano, Nergadze, Solomon G., Sullivan, Kevin F., Raimondi, Elena, Rocchi, Mariano, and Giulotto, Elena

Published
2015
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27. A G316A Polymorphism in the Ornithine Decarboxylase Gene Promoter Modulates MYCN-Driven Childhood Neuroblastoma

Author

Gamble, Laura D., primary, Purgato, Stefania, additional, Henderson, Michelle J., additional, Di Giacomo, Simone, additional, Russell, Amanda J., additional, Pigini, Paolo, additional, Murray, Jayne, additional, Valli, Emanuele, additional, Milazzo, Giorgio, additional, Giorgi, Federico M., additional, Cowley, Mark, additional, Ashton, Lesley J., additional, Bhalshankar, Jaydutt, additional, Schleiermacher, Gudrun, additional, Rihani, Ali, additional, Van Maerken, Tom, additional, Vandesompele, Jo, additional, Speleman, Frank, additional, Versteeg, Rogier, additional, Koster, Jan, additional, Eggert, Angelika, additional, Noguera, Rosa, additional, Stallings, Raymond L., additional, Tonini, Gian Paolo, additional, Fong, Kwun, additional, Vaksman, Zalman, additional, Diskin, Sharon J., additional, Maris, John M., additional, London, Wendy B., additional, Marshall, Glenn M., additional, Ziegler, David S., additional, Hogarty, Michael D., additional, Perini, Giovanni, additional, Norris, Murray D., additional, and Haber, Michelle, additional

Published
2021
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28. Erratum to: Centromere sliding on a mammalian chromosome

Author

Purgato, Stefania, Belloni, Elisa, Piras, Francesca M., Zoli, Monica, Badiale, Claudia, Cerutti, Federico, Mazzagatti, Alice, Perini, Giovanni, Della Valle, Giuliano, Nergadze, Solomon G., Sullivan, Kevin F., Raimondi, Elena, Rocchi, Mariano, and Giulotto, Elena

Published
2015
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29. Inhibition of polyamine synthesis and uptake reduces tumor progression and prolongs survival in mouse models of neuroblastoma

Author

Gamble, Laura D., Purgato, Stefania, Murray, Jayne, Xiao, Lin, Yu, Denise M. T., Hanssen, Kimberley M., Giorgi, Federico M., Carter, Daniel R., Gifford, Andrew J., Valli, Emanuele, Milazzo, Giorgio, Kamili, Alvin, Mayoh, Chelsea, Liu, Bing, Eden, Georgina, Sarraf, Sara, Allan, Sophie, Di Giacomo, Simone, Flemming, Claudia L., Russell, Amanda J., Cheung, Belamy B., Oberthuer, Andre, London, Wendy B., Fischer, Matthias, Trahair, Toby N., Fletcher, Jamie, I, Marshall, Glenn M., Ziegler, David S., Hogarty, Michael D., Burns, Mark R., Perini, Giovanni, Norris, Murray D., Haber, Michelle, Gamble, Laura D., Purgato, Stefania, Murray, Jayne, Xiao, Lin, Yu, Denise M. T., Hanssen, Kimberley M., Giorgi, Federico M., Carter, Daniel R., Gifford, Andrew J., Valli, Emanuele, Milazzo, Giorgio, Kamili, Alvin, Mayoh, Chelsea, Liu, Bing, Eden, Georgina, Sarraf, Sara, Allan, Sophie, Di Giacomo, Simone, Flemming, Claudia L., Russell, Amanda J., Cheung, Belamy B., Oberthuer, Andre, London, Wendy B., Fischer, Matthias, Trahair, Toby N., Fletcher, Jamie, I, Marshall, Glenn M., Ziegler, David S., Hogarty, Michael D., Burns, Mark R., Perini, Giovanni, Norris, Murray D., and Haber, Michelle

Abstract

Amplification of the MYCN oncogene is associated with an aggressive phenotype and poor outcome in childhood neuroblastoma. Polyamines are highly regulated essential cations that are frequently elevated in cancer cells, and the rate-limiting enzyme in polyamine synthesis, ornithine decarboxylase 1 (ODC1), is a direct transcriptional target of MYCN. Treatment of neuroblastoma cells with the ODC1 inhibitor difluoromethylornithine (DFMO), although a promising therapeutic strategy, is only partially effective at impeding neuroblastoma cell growth due to activation of compensatory mechanisms resulting in increased polyamine uptake from the surrounding microenvironment. In this study, we identified solute carrier family 3 member 2 (SLC3A2) as the key transporter involved in polyamine uptake in neuroblastoma. Knockdown of SLC3A2 in neuroblastoma cells reduced the uptake of the radiolabeled polyamine spermidine, and DFMO treatment increased SLC3A2 protein. In addition, MYCN directly increased polyamine synthesis and promoted neuroblastoma cell proliferation by regulating SLC3A2 and other regulatory components of the polyamine pathway. Inhibiting polyamine uptake with the small-molecule drug AMXT 1501, in combination with DFMO, prevented or delayed tumor development in neuroblastoma-prone mice and extended survival in rodent models of established tumors. Our findings suggest that combining AMXT 1501 and DFMO with standard chemotherapy might be an effective strategy for treating neuroblastoma.

Published
2019

30. Inhibition of polyamine synthesis and uptake reduces tumor progression and prolongs survival in mouse models of neuroblastoma

Author

Gamble, Laura D., primary, Purgato, Stefania, additional, Murray, Jayne, additional, Xiao, Lin, additional, Yu, Denise M. T., additional, Hanssen, Kimberley M., additional, Giorgi, Federico M., additional, Carter, Daniel R., additional, Gifford, Andrew J., additional, Valli, Emanuele, additional, Milazzo, Giorgio, additional, Kamili, Alvin, additional, Mayoh, Chelsea, additional, Liu, Bing, additional, Eden, Georgina, additional, Sarraf, Sara, additional, Allan, Sophie, additional, Di Giacomo, Simone, additional, Flemming, Claudia L., additional, Russell, Amanda J., additional, Cheung, Belamy B., additional, Oberthuer, Andre, additional, London, Wendy B., additional, Fischer, Matthias, additional, Trahair, Toby N., additional, Fletcher, Jamie I., additional, Marshall, Glenn M., additional, Ziegler, David S., additional, Hogarty, Michael D., additional, Burns, Mark R., additional, Perini, Giovanni, additional, Norris, Murray D., additional, and Haber, Michelle, additional

Published
2019
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31. The Hidden Genomic and Transcriptomic Plasticity of Giant Marker Chromosomes in Cancer

Author

Macchia, Gemma, primary, Severgnini, Marco, additional, Purgato, Stefania, additional, Tolomeo, Doron, additional, Casciaro, Hilen, additional, Cifola, Ingrid, additional, L’Abbate, Alberto, additional, Loverro, Anna, additional, Palumbo, Orazio, additional, Carella, Massimo, additional, Bianchini, Laurence, additional, Perini, Giovanni, additional, De Bellis, Gianluca, additional, Mertens, Fredrik, additional, Rocchi, Mariano, additional, and Storlazzi, Clelia Tiziana, additional

Published
2018
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33. Epigenetic origin of evolutionary novel centromeres

Author

Tolomeo, Doron, primary, Capozzi, Oronzo, additional, Stanyon, Roscoe R., additional, Archidiacono, Nicoletta, additional, D’Addabbo, Pietro, additional, Catacchio, Claudia R., additional, Purgato, Stefania, additional, Perini, Giovanni, additional, Schempp, Werner, additional, Huddleston, John, additional, Malig, Maika, additional, Eichler, Evan E., additional, and Rocchi, Mariano, additional

Published
2017
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34. MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

O'Brien, Rosemary, primary, Tran, Sieu L., additional, Maritz, Michelle F., additional, Liu, Bing, additional, Kong, Cheng Fei, additional, Purgato, Stefania, additional, Yang, Chen, additional, Murray, Jayne, additional, Russell, Amanda J., additional, Flemming, Claudia L., additional, von Jonquieres, Georg, additional, Pickett, Hilda A., additional, London, Wendy B., additional, Haber, Michelle, additional, Gunaratne, Preethi H., additional, Norris, Murray D., additional, Perini, Giovanni, additional, Fletcher, Jamie I., additional, and MacKenzie, Karen L., additional

Published
2016
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35. Abstract 488: High DKC1 expression supports neuroblastoma tumor cell proliferation and is strongly associated with poor patient outcomes

Author

O'Brien, Rosemary, primary, Maritz, Michelle F., additional, Kong, Cheng Fei, additional, Purgato, Stefania, additional, Liu, Bing, additional, Yang, Chen, additional, Russell, Amanda, additional, Murray, Jayne, additional, Flemming, Claudia, additional, Haber, Michelle, additional, Perini, Giovanni, additional, Norris, Murray D., additional, Fletcher, Jamie I., additional, and Mackenzie, Karen L., additional

Published
2015
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37. MAX to MYCN intracellular ratio drives the aggressive phenotype and clinical outcome of high risk neuroblastoma

Author

Simone Di Giacomo, Stefania Purgato, Sara Monticelli, Paolo Pigini, Michelle Haber, Daniela Erriquez, Roberto Bernardoni, Giorgio Milazzo, Murray D. Norris, Roberto Ciaccio, Giovanni Perini, Francesca Ferrucci, Ferrucci, Francesca, Ciaccio, Roberto, Monticelli, Sara, Pigini, Paolo, di Giacomo, Simone, Purgato, Stefania, Erriquez, Daniela, Bernardoni, Roberto, Norris, Murray, Haber, Michelle, Milazzo, Giorgio, and Perini, Giovanni

Subjects
0301 basic medicine, Biophysics, Intracellular Space, Context (language use), Aggressive phenotype, Apoptosis, Disease, MYCN gene amplification, Biochemistry, 03 medical and health sciences, Neuroblastoma, Structural Biology, Cell Line, Tumor, MYCN, Genetics, medicine, Humans, High risk neuroblastoma, Neoplasm Invasiveness, Gene Silencing, RNA, Messenger, neoplasms, Molecular Biology, Transcription factor, Neurons, N-Myc Proto-Oncogene Protein, business.industry, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors, Gene Amplification, Cell Differentiation, medicine.disease, Gene Expression Regulation, Neoplastic, 030104 developmental biology, HEK293 Cells, Phenotype, Treatment Outcome, Cancer research, business, Childhood Neuroblastoma, Intracellular, MAX
Abstract

Childhood neuroblastoma, a disease of the sympathetic nervous system, is the most common solid tumour of infancy, remarkably refractory to therapeutic treatments. One of the most powerful independent prognostic indicators for this disease is the amplification of the MYCN oncogene, which occurs at high levels in approximately 25% of neuroblastomas. Interestingly, amplification and not just expression of MYCN has a strong prognostic value, although this fact appears quite surprising as MYCN is a transcription factor that requires dimerising with its partner MAX, to exert its function. This observation greatly suggests that the role of MYCN in neuroblastoma should be examined in the context of MAX expression. In this report, we show that, in contrast to what is found in normal cells, MAX expression is significantly different among primary NBs, and that its level appears to correlate with the clinical outcome of the disease. Importantly, controlled modulation of MAX expression in neuroblastoma cells with different extents of MYCN amplification, demonstrates that MAX can instruct gene transcription programs that either reinforce or weaken the oncogenic process enacted by MYCN. In general, our work illustrates that it is the MAX to MYCN ratio that can account for tumour progression and clinical outcome in neuroblastoma and proposes that such a ratio should be considered as an important criterion to the design and development of anti-MYCN therapies.

Published
2017

38. Epigenetic origin of evolutionary novel centromeres

Author

Pietro D'Addabbo, Nicoletta Archidiacono, Claudia Rita Catacchio, Doron Tolomeo, Evan E. Eichler, Roscoe Stanyon, Werner Schempp, Maika Malig, Oronzo Capozzi, Mariano Rocchi, Stefania Purgato, John Huddleston, Giovanni Perini, Tolomeo, Doron, Capozzi, Oronzo, Stanyon, Roscoe R., Archidiacono, Nicoletta, D'Addabbo, Pietro, Catacchio, Claudia R., Purgato, Stefania, Perini, Giovanni, Schempp, Werner, Huddleston, John, Malig, Maika, Eichler, Evan E., and Rocchi, Mariano

Subjects
0301 basic medicine, Pongo abelii, neocentromere, Neocentromere, Satellite DNA, Centromere, polymorphisms of centromeres, DNA, Satellite, Biology, Genome, Article, Cell Line, Epigenesis, Genetic, Evolution, Molecular, 03 medical and health sciences, evolution of centromere, Animals, Humans, Epigenetics, Gene, Conserved Sequence, Chromosome 12, Segmental duplication, Multidisciplinary, neocentromeres, orangutan, chromosome rearrangements, BAC-FISH, 030104 developmental biology, Evolutionary biology
Abstract

Most evolutionary new centromeres (ENC) are composed of large arrays of satellite DNA and surrounded by segmental duplications. However, the hypothesis is that ENCs are seeded in an anonymous sequence and only over time have acquired the complexity of “normal” centromeres. Up to now evidence to test this hypothesis was lacking. We recently discovered that the well-known polymorphism of orangutan chromosome 12 was due to the presence of an ENC. We sequenced the genome of an orangutan homozygous for the ENC, and we focused our analysis on the comparison of the ENC domain with respect to its wild type counterpart. No significant variations were found. This finding is the first clear evidence that ENC seedings are epigenetic in nature. The compaction of the ENC domain was found significantly higher than the corresponding WT region and, interestingly, the expression of the only gene embedded in the region was significantly repressed.

Published
2017
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39. MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin

Author

Georg von Jonquieres, Murray D. Norris, Giovanni Perini, Sieu L. Tran, Jamie I. Fletcher, Rosemary O'Brien, Wendy B. London, Michelle Haber, Hilda A. Pickett, Chen Yang, Cheng Fei Kong, Michelle F. Maritz, Jayne Murray, Claudia Flemming, Karen L. MacKenzie, Amanda J. Russell, Stefania Purgato, Bing Liu, Preethi H. Gunaratne, O'Brien, R, Tran, SL, Maritz, MF, Liu, B, Kong, CF, Purgato, S, Yang, C, Murray, J, Russell, AJ, Flemming, CL, Von Jonquieres, G, Pickett, HA, London, WB, Haber, M, Gunaratne, PH, Norris, MD, Perini, G, Fletcher, JI, MacKenzie, KL, O'Brien, Rosemary, Tran, Sieu L., Maritz, Michelle F., Liu, Bing, Kong, Cheng Fei, Purgato, Stefania, Yang, Chen, Murray, Jayne, Russell, Amanda J., Flemming, Claudia L., Von Jonquieres, Georg, Pickett, Hilda A., London, Wendy B., Haber, Michelle, Gunaratne, Preethi H., Norris, Murray D., Perini, Giovanni, Fletcher, Jamie I., and Mackenzie, Karen L

Subjects
0301 basic medicine, Telomerase, Cancer Research, Ribosome biogenesis, Cell Cycle Proteins, Biology, Dyskerin, Proto-Oncogene Proteins c-myc, 03 medical and health sciences, Telomerase RNA component, Neuroblastoma, 0302 clinical medicine, Downregulation and upregulation, Ribosomal protein, medicine, Humans, functions and broader implications, RNA-binding protein dyskerin, Cells, Cultured, RNA, Nuclear Proteins, medicine.disease, G1 Phase Cell Cycle Checkpoints, 030104 developmental biology, Oncology, 030220 oncology & carcinogenesis, Cancer research, Tumor Suppressor Protein p53, DKC1 gene, Ribosomes
Abstract

The RNA-binding protein dyskerin, encoded by the DKC1 gene, functions as a core component of the telomerase holoenzyme as well as ribonuclear protein complexes involved in RNA processing and ribosome biogenesis. The diverse roles of dyskerin across many facets of RNA biology implicate its potential contribution to malignancy. In this study, we examined the expression and function of dyskerin in neuroblastoma. We show that DKC1 mRNA levels were elevated relative to normal cells across a panel of 15 neuroblastoma cell lines, where both N-Myc and c-Myc directly targeted the DKC1 promoter. Upregulation of MYCN was shown to dramatically increase DKC1 expression. In two independent neuroblastoma patient cohorts, high DKC1 expression correlated strongly with poor event-free and overall survival (P < 0.0001), independently of established prognostic factors. RNAi-mediated depletion of dyskerin inhibited neuroblastoma cell proliferation, including cells immortalized via the telomerase-independent ALT mechanism. Furthermore, dyskerin attenuation impaired anchorage-independent proliferation and tumor growth. Overexpression of the telomerase RNA component, hTR, demonstrated that this proliferative impairment was not a consequence of telomerase suppression. Instead, ribosomal stress, evidenced by depletion of small nucleolar RNAs and nuclear dispersal of ribosomal proteins, was the likely cause of the proliferative impairment in dyskerin-depleted cells. Accordingly, dyskerin suppression caused p53-dependent G1 cell-cycle arrest in p53 wild-type cells, and a p53-independent pathway impaired proliferation in cells with p53 dysfunction. Together, our findings highlight dyskerin as a new therapeutic target in neuroblastoma with crucial telomerase-independent functions and broader implications for the spectrum of malignancies driven by MYC family oncogenes. Cancer Res; 76(12); 3604–17. ©2016 AACR.

Published
2015

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