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594 results on '"Corben P"'

1. Phosphorylation-driven epichaperome assembly is a regulator of cellular adaptability and proliferation.

Author

Roychowdhury, Tanaya, McNutt, Seth, Pasala, Chiranjeevi, Nguyen, Hieu, Thornton, Daniel, Sharma, Sahil, Botticelli, Luke, Digwal, Chander, Joshi, Suhasini, Yang, Nan, Panchal, Palak, Chakrabarty, Souparna, Bay, Sadik, Markov, Vladimir, Kwong, Charlene, Lisanti, Jeanine, Chung, Sun, Ginsberg, Stephen, Yan, Pengrong, De Stanchina, Elisa, Corben, Adriana, Modi, Shanu, Alpaugh, Mary, Colombo, Giorgio, Erdjument-Bromage, Hediye, Neubert, Thomas, Chalkley, Robert, Baker, Peter, Burlingame, Alma, Rodina, Anna, Chiosis, Gabriela, and Chu, Feixia

Subjects
Humans, Cell Proliferation, Phosphorylation, HSP90 Heat-Shock Proteins, Molecular Chaperones, Protein Processing, Post-Translational, Animals, Protein Interaction Maps, Mice, Serine, Cell Line, Tumor
Abstract

The intricate network of protein-chaperone interactions is crucial for maintaining cellular function. Recent discoveries have unveiled the existence of specialized chaperone assemblies, known as epichaperomes, which serve as scaffolding platforms that orchestrate the reconfiguration of protein-protein interaction networks, thereby enhancing cellular adaptability and proliferation. This study explores the structural and regulatory aspects of epichaperomes, with a particular focus on the role of post-translational modifications (PTMs) in their formation and function. A key finding is the identification of specific PTMs on HSP90, particularly at residues Ser226 and Ser255 within an intrinsically disordered region, as critical determinants of epichaperome assembly. Our data demonstrate that phosphorylation of these serine residues enhances HSP90s interactions with other chaperones and co-chaperones, creating a microenvironment conducive to epichaperome formation. Moreover, we establish a direct link between epichaperome function and cellular physiology, particularly in contexts where robust proliferation and adaptive behavior are essential, such as in cancer and pluripotent stem cell maintenance. These findings not only provide mechanistic insights but also hold promise for the development of novel therapeutic strategies targeting chaperone assemblies in diseases characterized by epichaperome dysregulation, thereby bridging the gap between fundamental research and precision medicine.

Published
2024

4. Coronal Heating as Determined by the Solar Flare Frequency Distribution Obtained by Aggregating Case Studies

Author

Mason, James Paul, Werth, Alexandra, West, Colin G., Youngblood, Allison A., Woodraska, Donald L., Peck, Courtney, Lacjak, Kevin, Frick, Florian G., Gabir, Moutamen, Alsinan, Reema A., Jacobsen, Thomas, Alrubaie, Mohammad, Chizmar, Kayla M., Lau, Benjamin P., Dominguez, Lizbeth Montoya, Price, David, Butler, Dylan R., Biron, Connor J., Feoktistov, Nikita, Dewey, Kai, Loomis, N. E., Bodzianowski, Michal, Kuybus, Connor, Dietrick, Henry, Wolfe, Aubrey M., Guerrero, Matt, Vinson, Jessica, Starbuck, Peter, Litton, Shelby D, Beck, M. G., Fisch, Jean-Paul, West, Ayana, Muniz, Alexis A., Chavez, Luis, Upthegrove, Zachary T., Runyon, Brenton M., Salazar, J., Kritzberg, Jake E., Murrel, Tyler, Ho, Ella, LaFemina, Quintin Y., Elbashir, Sara I., Chang, Ethan C., Hudson, Zachary A., Nussbaum, Rosemary O., Kennedy, Kellen, Kim, Kevin, Arango, Camila Villamil, Albakr, Mohammed A., Rotter, Michael, Garscadden, A. J., Salcido-Alcontar JR, Antonio, Pearl, Harrison M., Stepaniak, Tyler, Marquez, Josie A., Marsh, Lauren, Andringa, Jesse C, Osogwin, Austin, Shields, Amanda M., Brookins, Sarah, Hach, Grace K., Clausi, Alexis R., Millican, Emily B., Jaimes, Alan A, Graham, Alaina S., Burritt, John J., Perez, J. S., Ramirez, Nathaniel, Suri, Rohan, Myer, Michael S., Kresek, Zoe M., Goldsberry, C. A., Payne, Genevieve K., Jourabchi, Tara, Hu, J., Lucca, Jeffrey, Feng, Zitian, Gilpatrick, Connor B., Khan, Ibraheem A., Warble, Keenan, Sweeney, Joshua D., Dorricott, Philip, Meyer, Ethan, Kothamdi, Yash S., Sohail, Arman S., Grell, Kristyn, Floyd, Aidan, Bard, Titus, Mathieson, Randi M., Reed, Joseph, Cisneros, Alexis, Payne, Matthew P., Jarriel, J. R., Mora, Jacqueline Rodriguez, Sundell, M. E., Patel, Kajal, Alesmail, Mohammad, Alnasrallah, Yousef A, Abdullah, Jumana T., Molina-Saenz, Luis, Tayman, K. E., Brown, Gabriel T., Kerr-Layton, Liana, Berriman-Rozen, Zachary D., Hiatt, Quinn, Kalra, Etash, Ong, Jason, Vadayar, Shreenija, Shannahan, Callie D., Benke, Evan, zhang, Jinhua, Geisman, Jane, Martyr, Cara, Ameijenda, Federico, Akruwala, Ushmi H., Nehring, Molly, Kissner, Natalie, Rule, Ian C., Learned, Tyler, Smith, Alexandra N., Mazzotta, Liam, Rounsefell, Tyndall, Eyeson, Elizabeth A., Shelby, Arlee K., Moll, Tyler S, Menke, Riley, Shahba, Hannan, House Jr., Tony A., Clark, David B., Burns, Annemarie C., de La Beaujardiere, Tristan, Trautwein, Emily D., Plantz, Will, Reeves, Justin, Faber, Ian, Buxton, B. W., Highhouse, Nigel, Landrey, Kalin, Hansen, Connor M, Chen, Kevin, Hales, Ryder Buchanan, Borgerding, Luke R., Guo, Mutian, Crow, Christian J., Whittall, Lloyd C., Simmons, Conor, Folarin, Adeduni, Parkinson, Evan J., Rahn, Anna L., Blevins, Olivia, Morelock, Annalise M., Kelly, Nicholas, Parker, Nathan L., Smith, Kelly, Plzak, Audrey E., Saeb, David, Hares, Cameron T., Parker, Sasha R., McCoy, Andrew, Pham, Alexander V., Lauzon, Megan, Kennedy, Cayla J., Reyna, Andrea B., Acosta, Daniela M. Meza, Cool, Destiny J., Steinbarth, Sheen L., Mendoza-Anselmi, Patricia, Plutt, Kaitlyn E., Kipp, Isabel M, Rakhmonova, M., Brown, Cameron L., Van Anne, Gabreece, Moss, Alexander P., Golden, Olivia, Kirkpatrick, Hunter B., Colleran, Jake R., Sullivan, Brandon J, Tran, Kevin, Carpender, Michael Andrew, Mundy, Aria T., Koenig, Greta, Oudakker, Jessica, Engelhardt, Rasce, Ales, Nolan, Wexler, Ethan Benjamin, Beato, Quinn I, Chen, Lily, Cochran, Brooke, Hill, Paula, Hamilton, Sean R., Hashiro, Kyle, Khan, Usman, Martinez, Alexa M., Brockman, Jennifer L., Mallory, Macguire, Reed, Charlie, Terrile, Richard, Singh, Savi, Watson, James Adam, Creany, Joshua B., Price, Nicholas K., Miften, Aya M., Tran, Bryn, Kamenetskiy, Margaret, Martinez, Jose R., Opp, Elena N., Huang, Jianyang, Fails, Avery M., Belei, Brennan J., Slocum, Ryan, Astalos, Justin, East, Andrew, Nguyen, Lena P., Pherigo, Callie C, East, Andrew N., Li, David Y., Nelson, Maya LI, Taylor, Nicole, Odbayar, Anand, Rives, Anna Linnea, Mathur, Kabir P., Billingsley, Jacob, Polikoff, Hyden, Driscoll, Michael, Wilson, Orion K., Lahmers, Kyle, Toon, Nathaniel J., Lippincott, Sam, Musgrave, Andrew J., Gregory, Alannah H., Pitsuean-Meier, Sedique, Jesse, Trevor, Smith, Corey, Miles, Ethan J., Kainz, Sabrina J. H. T., Ji, Soo Yeun, Nguyen, Lena, Aryan, Maryam, Dinser, Alexis M., Shortman, Jadon, Bastias, Catalina S, Umbricht, Thomas D, Cage, Breonna, Randolph, Parker, Pollard, Matthew, Simone, Dylan M., Aramians, Andrew, Brecl, Ariana E., Robert, Amanda M., Zenner, Thomas, Saldi, Maxwell, Morales, Gavin, Mendez, Citlali, Syed, Konner, Vogel, Connor Maklain, Cone, Rebecca A., Berhanu, Naomi, Carpenter, Emily, Leoni, Cecilia, Bryan, Samuel, Ramachandra, Nidhi, Shaw, Timothy, Lee, E. C., Monyek, Eli, Wegner, Aidan B., Sharma, Shajesh, Lister, Barrett, White, Jamison R., Willard, John S., Sulaiman, S. A, Blandon, Guillermo, Narayan, Anoothi, Ruger, Ryan, Kelley, Morgan A., Moreno, Angel J., Balcer, Leo M, Ward-Chene, N. R. D., Shelby, Emma, Reagan, Brian D., Marsh, Toni, Sarkar, Sucheta, Kelley, Michael P., Fell, Kevin, Balaji, Sahana, Hildebrand, Annalise K., Shoha, Dominick, Nandu, Kshmya, Tucker, Julia, Cancio, Alejandro R., Wang, Jiawei, Rapaport, Sarah Grace, Maravi, Aimee S., Mayer, Victoria A., Miller, Andrew, Bence, Caden, Koke, Emily, Fauntleroy, John T, Doermer, Timothy, Al-Ghazwi, Adel, Morgan, Remy, Alahmed, Mohammed S., Mathavan, Adam Izz Khan Mohd Reduan, Silvester, H. K., Weiner, Amanda M., Liu, Nianzi, Iovan, Taro, Jensen, Alexander V., AlHarbi, Yazeed A., Jiang, Yufan, Zhang, Jiaqi, Jones, Olivia M., Huang, Chenqi, Reh, Eileen N., Alhamli, Dania, Pettine, Joshua, Zhou, Chongrui, Kriegman, Dylan, Yang, Jianing, Ash, Kevin, Savage, Carl, Kaiser, Emily, Augenstein, Dakota N., Padilla, Jacqueline, Stark, Ethan K., Hansen, Joshua A., Kokes, Thomas, Huynh, Leslie, Sanchez-Sanchez, Gustavo, Jeseritz, Luke A., Carillion, Emma L., Vepa, Aditya V., Khanal, Sapriya, Behr, Braden, Martin, Logan S., McMullan, Jesse J., Zhao, Tianwei, Williams, Abigail K., Alqabani, Emeen, Prinster, Gale H., Horne, Linda, Ruggles-Delgado, Kendall, Otto, Grant, Gomez, Angel R., Nguyen, Leonardo, Brumley, Preston J., Venegas, Nancy Ortiz, Varela, Ilian, Brownlow, Jordi, Cruz, Avril, Leiker, Linzhi, Batra, Jasleen, Hutabarat, Abigail P., Nunes-Valdes, Dario, Jameson, Connor, Naqi, Abdulaziz, Adams, Dante Q., Biediger, Blaine B., Borelli, William T, Cisne, Nicholas A., Collins, Nathaniel A., Curnow, Tyler L., Gopalakrishnan, Sean, Griffin, Nicholas F., Herrera, Emanuel, McGarvey, Meaghan V., Mellett, Sarah, Overchuk, Igor, Shaver, Nathan, Stratmeyer, Cooper N., Vess, Marcus T., Juels, Parker, Alyami, Saleh A., Gale, Skylar, Wallace, Steven P., Hunter, Samuel C, Lonergan, Mia C., Stewart, Trey, Maksimuk, Tiffany E., Lam, Antonia, Tressler, Judah, Napoletano, Elena R., Miller, Joshua B., Roy, Marc G., Chanders, Jasey, Fischer, Emmalee, Croteau, A. J., Kuiper, Nicolas A., Hoffman, Alex, DeBarros, Elyse, Curry, Riley T., Brzostowicz, A., Courtney, Jonas, Zhao, Tiannie, Szabo, Emi, Ghaith, Bandar Abu, Slyne, Colin, Beck, Lily, Quinonez, Oliver, Collins, Sarah, Madonna, Claire A., Morency, Cora, Palizzi, Mallory, Herwig, Tim, Beauprez, Jacob N., Ghiassi, Dorsa, Doran, Caroline R., Yang, Zhanchao, Padgette, Hannah M., Dicken, Cyrus A., Austin, Bryce W., Phalen, Ethan J., Xiao, Catherine, Palos, Adler, Gerhardstein, Phillip, Altenbern, Ava L., Orbidan, Dan, Dorr, Jackson A., Rivas, Guillermo A., Ewing, Calvin A, Giebner, B. C., McEntee, Kelleen, Kite, Emily R., Crocker, K. A., Haley, Mark S., Lezak, Adrienne R., McQuaid, Ella, Jeong, Jacob, Albaum, Jonathan, Hrudka, E. M., Mulcahy, Owen T., Tanguma, Nolan C., Oishi-Holder, Sean, White, Zachary, Coe, Ryan W., Boyer, Christine, Chapman, Mitchell G., Fortino, Elise, Salgado, Jose A., Hellweg, Tim, Martinez, Hazelia K., Mitchell, Alexander J., Schubert, Stephanie H., Schumacher, Grace K, Tesdahl, Corey D, Uphoff, C. H., Vassilyev, Alexandr, Witkoff, Briahn, Wolle, Jackson R., Dice, Kenzie A., Behrer, Timothy A., Bowen, Troy, Campbell, Andrew J, Clarkson, Peter C, Duong, Tien Q., Hawat, Elijah, Lopez, Christian, Olson, Nathaniel P., Osborn, Matthew, Peou, Munisettha E., Vaver, Nicholas J., Husted, Troy, Kallemeyn, Nicolas Ian, Spangler, Ava A, Mccurry, Kyle, Schultze, Courtney, Troisi, Thomas, Thomas, Daniel, Ort, Althea E., Singh, Maya A., Soon, Caitlin, Patton, Catherine, Billman, Jayce A., Jarvis, Sam, Hitt, Travis, Masri, Mirna, Albalushi, Yusef J., Schofer, Matthew J, Linnane, Katherine B., Knott, Philip Whiting, Valencia, Whitney, Arias-Robles, Brian A., Ryder, Diana, Simone, Anna, Abrams, Jonathan M., Belknap, Annelene L., Rouse, Charlotte, Reynolds, Alexander, Petric, Romeo S. L., Gomez, Angel A., Meiselman-Ashen, Jonah B., Carey, Luke, Dias, John S., Fischer-White, Jules, Forbes, Aidan E., Galarraga, Gabriela, Kennedy, Forrest, Lawlor, Rian, Murphy, Maxwell J., Norris, Cooper, Quarderer, Josh, Waller, Caroline, Weber, Robert J., Gunderson, Nicole, Boyne, Tom, Gregory, Joshua A., Propper, Henry Austin, von Peccoz, Charles B. Beck, Branch, Donovan, Clarke, Evelyn, Cutler, Libby, Dabberdt, Frederick M., Das, Swagatam, Figueirinhas, John Alfred D., Fougere, Benjamin L., Roy, Zoe A., Zhao, Noah Y., Cox, Corben L., Barnhart, Logan D. W., Craig, Wilmsen B., Moll, Hayden, Pohle, Kyle, Mueller, Alexander, Smith, Elena K., Spicer, Benjamin C., Aycock, Matthew C., Bat-Ulzii, Batchimeg, Murphy, Madalyn C., Altokhais, Abdullah, Thornally, Noah R., Kleinhaus, Olivia R., Sarfaraz, Darian, Barnes, Grant M., Beard, Sara, Banda, David J, Davis, Emma A. B., Huebsch, Tyler J., Wagoner, Michaela, Griego, Justus, Hale, Jack J. Mc, Porter, Trevor J., Abrashoff, Riley, Phan, Denise M., Smith, Samantha M., Srivastava, Ashish, Schlenker, Jared A. W., Madsen, Kasey O., Hirschmann, Anna E., Rankin, Frederick C, Akbar, Zainab A., Blouin, Ethan, Coleman-Plante, Aislinn, Hintsa, Evan, Lookhoff, Emily, Amer, Hamzi, Deng, Tianyue, Dvorak, Peter, Minimo, Josh, Plummer, William C., Ton, Kelly, Solt, Lincoln, AlAbbas, Batool H., AlAwadhi, Areej A., Cooper, Nicholas M., Corbitt, Jessica S, Dunlap, Christian, Johnson, Owen, Malone, Ryan A., Tellez, Yesica, Wallace, Logan, Ta, Michael-Tan D., Wheeler, Nicola H., Ramirez, Ariana C., Huang, Shancheng, Mehidic, Amar, Christiansen, Katherine E, Desai, Om, Domke, Emerson N., Howell, Noah H., Allsbrook, Martin, Alnaji, Teeb, England, Colin, Siles, Nathan, Burton, Nicholas David, Cruse, Zoe, Gilmartin, Dalton, Kim, Brian T., Hattendorf, Elsie, Buhamad, Maryam, Gayou, Lily, Seglem, Kasper, Alkhezzi, Tameem, Hicks, Imari R., Fife, Ryann, Pelster, Lily M., Fix, Alexander, Sur, Sohan N., Truong, Joshua K., Kubiak, Bartlomiej, Bondar, Matthew, Shi, Kyle Z., Johnston, Julia, Acevedo, Andres B., Lee, Junwon, Solorio, William J., Johnston, Braedon Y., McCormick, Tyler, Olguin, Nicholas, Pastor, Paige J., Wilson, Evan M., Trunko, Benjamin L., Sjoroos, Chris, Adams, Kalvyn N, Bell, Aislyn, Brumage-Heller, Grant, Canales, Braden P., Chiles, Bradyn, Driscoll, Kailer H., Hill, Hallie, Isert, Samuel A., Ketterer, Marilyn, Kim, Matthew M., Mewhirter, William J., Phillips, Lance, Phommatha, Krista, Quinn, Megan S., Reddy, Brooklyn J., Rippel, Matthew, Russell, Bowman, Williams, Sajan, Pixley, Andrew M., Gapin, Keala C., Peterson, B., Ruprecht, Collin, Hardie, Isabelle, Li, Isaac, Erickson, Abbey, Gersabeck, Clint, Gopalani, Mariam, Allanqawi, Nasser, Burton, Taylor, Cahn, Jackson R., Conti, Reese, White, Oliver S., Rojec, Stewart, Hogen, Blake A., Swartz, Jason R., Dick, R., Battist, Lexi, Dunn, Gabrielle M., Gasser, Rachel, Logan, Timothy W., Sinkovic, Madeline, Schaller, Marcus T., Heintz, Danielle A., Enrich, Andrew, Sanchez, Ethan S., Perez, Freddy, Flores, Fernando, Kapla, Shaun D., Shockley, Michael C., Phillips, Justin, Rumley, Madigan, Daboub, Johnston, Karsh, Brennan J., Linders, Bridget, Chen, Sam, Do, Helen C., Avula, Abhinav, French, James M., Bertuccio, Chrisanna, Hand, Tyler, Lee, Adrianna J., Neeland, Brenna K, Salazar, Violeta, Andrew, Carter, Barmore, Abby, Beatty, Thomas, Alonzi, Nicholas, Brown, Ryan, Chandler, Olivia M., Collier, Curran, Current, Hayden, Delasantos, Megan E., Bonilla, Alberto Espinosa de los Monteros, Fowler, Alexandra A., Geneser, Julianne R., Gentry, Eleanor, Gustavsson, E. R., Hansson, Jonathan, Hao, Tony Yunfei, Herrington, Robert N., Kelly, James, Kelly, Teagan, Kennedy, Abigail, Marquez, Mathew J., Meillon, Stella, Palmgren, Madeleine L., Pesce, Anneliese, Ranjan, Anurag, Robertson, Samuel M., Smith, Percy, Smith, Trevor J, Soby, Daniel A., Stratton, Grant L., Thielmann, Quinn N., Toups, Malena C., Veta, Jenna S., Young, Trenton J., Maly, Blake, Manzanares, Xander R., Beijer, Joshua, George, Jacob D., Mills, Dylan P., Ziebold, Josh J, Chambers, Paige, Montoya, Michael, Cheang, Nathan M., Anderson, Hunter J., Duncan, Sheridan J., Ehrlich, Lauren, Hudson, Nathan C., Kiechlin, Jack L., Koch, Will, Lee, Justin, Menassa, Dominic, Oakes, S. H., Petersen, Audrey J., Bunsow, J. R. Ramirez, Bay, Joshua, Ramirez, Sacha, Fenwick, Logan D., Boyle, Aidan P., Hibbard, Lea Pearl, Haubrich, Calder, Sherry, Daniel P., Jenkins, Josh, Furney, Sebastian, Velamala, Anjali A., Krueger, Davis J., Thompson, William N., Chhetri, Jenisha, Lee, Alexis Ying-Shan, Ray, Mia G. V., Recchia, John C., Lengerich, Dylan, Taulman, Kyle, Romero, Andres C., Steward, Ellie N., Russell, Sloan, Hardwick, Dillon F., Wootten, Katelynn, Nguyen, Valerie A., Quispe, Devon, Ragsdale, Cameron, Young, Isabel, Atchley-Rivers, N. S., Stribling, Jordin L., Gentile, Julia G, Boeyink, Taylor A., Kwiatkowski, Daniel, Dupeyron, Tomi Oshima, Crews, Anastasia, Shuttleworth, Mitchell, Dresdner, Danielle C., Flackett, Lydia, Haratsaris, Nicholas, Linger, Morgan I, Misener, Jay H., Patti, Samuel, Pine, Tawanchai P., Marikar, Nasreen, Matessi, Giorgio, Routledge, Allie C., Alkaabi, Suhail, Bartman, Jessica L., Bisacca, Gabrielle E., Busch, Celeste, Edwards, Bree, Staudenmier, Caitlyn, Starling, Travis, McVey, Caden, Montano, Maximus, Contizano, Charles J., Taylor, Eleanor, McIntyre, James K., Victory, Andrew, McCammon, Glen S., Kimlicko, Aspen, Sheldrake, Tucker, Shelchuk, Grace, Von Reich, Ferin J., Hicks, Andrew J., O'neill, Ian, Rossman, Beth, Taylor, Liam C., MacDonald, William, Becker, Simone E., Han, Soonhee, O'Sullivan, Cian, Wilcove, Isaac, Brennan, David J., Hanley, Luke C., Hull, Owen, Wilson, Timothy R., Kalmus, Madison H., Berv, Owen A., Harris, Logan Swous, Doan, Chris H, Londres, Nathan, Parulekar, Anish, Adam, Megan M., Angwin, Abigail, Cabbage, Carter C., Colleran, Zachary, Pietras, Alex, Seux, Octave, Oros, Ryan, Wilkinson, Blake C., Nguyen, Khoa D, Trank-Greene, Maedee, Barone, Kevin M., Snyder, G. L., Biehle, Samuel J, Billig, Brennen, Almquist, Justin Thomas, Dixon, Alyssa M., Erickson, Benjamin, Evans, Nathan, Genne, SL, Kelly, Christopher M, Marcus, Serafima M., Ogle, Caleb, Patel, Akhil, Vendetti, Evan, Courtney, Olivia, Deel, Sean, Del Foco, Leonardo, Gjini, Michael, Haines, Jessica, Hoff, Isabelle J., Jones, M. R., Killian, Dominic, Kuehl, Kirsten, Kuester, Chrisanne, Lantz, Maxwell B., Lee, Christian J, Mauer, Graham, McKemey, Finbar K., Millican, Sarah J., Rosasco, Ryan, Stewart, T. C., VanEtten, Eleanor, Derwin, Zachary, Serio, Lauren, Sickler, Molly G., Blake, Cassidy A., Patel, Neil S., Fox, Margaret, Gray, Michael J, Ziegler, Lucas J., Kumar, Aman Priyadarshi, Polly, Madelyn, Mesgina, Sarah, McMorris, Zane, Griffin, Kyle J., Haile, L. N., Bassel, Claire, Dixon, Thomas J., Beattie, Ryan, Houck, Timothy J, Rodgers, Maeve, Trofino, Tyson R., Lukianow, Dax, Smart, Korben, Hall, Jacqueline L., Bone, Lauren, Baldwin, James O., Doane, Connor, Almohsen, Yousef A., Stamos, Emily, Acha, Iker, Kim, Jake, Samour II, Antonio E., Chavali, S., Kanokthippayakun, Jeerakit, Gotlib, Nicholas, Murphy, Ryan C., Archibald, Jack. W., Brimhall, Alexander J, Boyer, Aidan, Chapman, Logan T., Chadda, Shivank, Sibrell, Lisa, Vallery, Mia M., Conroy, Thomas C., Pan, Luke J., Balajonda, Brian, Fuhrman, Bethany E. S., Alkubaisi, Mohamed, Engelstad, Jacob, Dodrill, Joshua, Fuchs, Calvin R., Bullard-Connor, Gigi, Alhuseini, Isehaq, Zygmunt, James C., Sipowicz, Leo, Hayrynen, Griffin A., McGill, Riley M., Keating, Caden J., Hart, Omer, Cyr, Aidan St., Steinsberger, Christopher H., Thoman, Gerig, Wood, Travis M., Ingram, Julia A., Dominguez, J., Georgiades, Nathaniel James, Johnson, Matthew, Johnson, Sawyer, Pedersen, Alexander J., Ralapanawe, Anoush K, Thomas, Jeffrey J., Sato, Ginn A., Reynolds, Hope, Nasser, Liebe, Mizzi, Alexander Z., Damgaard, Olivia, Baflah, Abdulrahman A., Liu, Steven Y., Salindeho, Adam D., Norden, Kelso, Gearhart, Emily E., Krajnak, Zack, Szeremeta, Philip, Amos, Meggan, Shin, Kyungeun, Muckenthaler, Brandon A., Medialdea, Melissa, Beach, Simone, Wilson, Connor B., Adams, Elena R, Aldhamen, Ahmed, Harris, Coyle M., Hesse, Troy M., Golding, Nathan T., Larter, Zachary, Hernandez, Angel, Morales, Genaro, Traxler, Robert B., Alosaimi, Meshal, Fitton, Aidan F., Aaron, James Holland, Lee, Nathaniel F., Liao, Ryan Z., Chen, Judy, French, Katherine V., Loring, Justin, Colter, Aurora, McConvey, Rowan, Colozzi, Michael, Vann, John D., Scheck, Benjamin T., Weigand, Anthony A, Alhabeeb, Abdulelah, Idoine, Yolande, Woodard, Aiden L., Medellin, Mateo M., Ratajczyk, Nicholas O, Tobin, Darien P., Collins, Jack C., Horning, Thomas M., Pellatz, Nick, Pitten, John, Lordi, Noah, Patterson, Alyx, Hoang, Thi D, Zimmermann, Ingrid H, Wang, Hongda, Steckhahn, Daniel, Aradhya, Arvind J., Oliver, Kristin A., Cai, Yijian, Wang, Chaoran, Yegovtsev, Nikolay, Wu, Mengyu, Ganesan, Koushik, Osborne, Andrew, Wickenden, Evan, Meyer, Josephine C., Chaparro, David, Visal, Aseem, Liu, Haixin, Menon, Thanmay S., Jin, Yan, Wilson, John, Erikson, James W., Luo, Zheng, Shitara, Nanako, Nelson, Emma E, Geerdts, T. R., Ortiz, Jorge L Ramirez, and Lewandowski, H. J.

Subjects
Astrophysics - Solar and Stellar Astrophysics
Abstract

Flare frequency distributions represent a key approach to addressing one of the largest problems in solar and stellar physics: determining the mechanism that counter-intuitively heats coronae to temperatures that are orders of magnitude hotter than the corresponding photospheres. It is widely accepted that the magnetic field is responsible for the heating, but there are two competing mechanisms that could explain it: nanoflares or Alfv\'en waves. To date, neither can be directly observed. Nanoflares are, by definition, extremely small, but their aggregate energy release could represent a substantial heating mechanism, presuming they are sufficiently abundant. One way to test this presumption is via the flare frequency distribution, which describes how often flares of various energies occur. If the slope of the power law fitting the flare frequency distribution is above a critical threshold, $\alpha=2$ as established in prior literature, then there should be a sufficient abundance of nanoflares to explain coronal heating. We performed $>$600 case studies of solar flares, made possible by an unprecedented number of data analysts via three semesters of an undergraduate physics laboratory course. This allowed us to include two crucial, but nontrivial, analysis methods: pre-flare baseline subtraction and computation of the flare energy, which requires determining flare start and stop times. We aggregated the results of these analyses into a statistical study to determine that $\alpha = 1.63 \pm 0.03$. This is below the critical threshold, suggesting that Alfv\'en waves are an important driver of coronal heating., Comment: 1,002 authors, 14 pages, 4 figures, 3 tables, published by The Astrophysical Journal on 2023-05-09, volume 948, page 71

Published
2023
Full Text
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5. Clinical management guidelines for Friedreich ataxia: best practice in rare diseases.

Author

Corben, Louise, Collins, Veronica, Milne, Sarah, Farmer, Jennifer, Musheno, Ann, Lynch, David, Subramony, Sub, Pandolfo, Massimo, Schulz, Jörg, Lin, Kim, and Delatycki, Martin

Subjects
Evidence, Friedreich ataxia, GRADE, Guidelines, Recommendations, Humans, Friedreich Ataxia, Rare Diseases
Abstract

BACKGROUND: Individuals with Friedreich ataxia (FRDA) can find it difficult to access specialized clinical care. To facilitate best practice in delivering healthcare for FRDA, clinical management guidelines (CMGs) were developed in 2014. However, the lack of high-certainty evidence and the inadequacy of accepted metrics to measure health status continues to present challenges in FRDA and other rare diseases. To overcome these challenges, the Grading of Recommendations Assessment and Evaluation (GRADE) framework for rare diseases developed by the RARE-Bestpractices Working Group was adopted to update the clinical guidelines for FRDA. This approach incorporates additional strategies to the GRADE framework to support the strength of recommendations, such as review of literature in similar conditions, the systematic collection of expert opinion and patient perceptions, and use of natural history data. METHODS: A panel representing international clinical experts, stakeholders and consumer groups provided oversight to guideline development within the GRADE framework. Invited expert authors generated the Patient, Intervention, Comparison, Outcome (PICO) questions to guide the literature search (2014 to June 2020). Evidence profiles in tandem with feedback from individuals living with FRDA, natural history registry data and expert clinical observations contributed to the final recommendations. Authors also developed best practice statements for clinical care points that were considered self-evident or were not amenable to the GRADE process. RESULTS: Seventy clinical experts contributed to fifteen topic-specific chapters with clinical recommendations and/or best practice statements. New topics since 2014 include emergency medicine, digital and assistive technologies and a stand-alone section on mental health. Evidence was evaluated according to GRADE criteria and 130 new recommendations and 95 best practice statements were generated. DISCUSSION AND CONCLUSION: Evidence-based CMGs are required to ensure the best clinical care for people with FRDA. Adopting the GRADE rare-disease framework enabled the development of higher quality CMGs for FRDA and allows individual topics to be updated as new evidence emerges. While the primary goal of these guidelines is better outcomes for people living with FRDA, the process of developing the guidelines may also help inform the development of clinical guidelines in other rare diseases.

Published
2022

6. Natural History of Friedreich Ataxia: Heterogeneity of Neurologic Progression and Consequences for Clinical Trial Design.

Author

Rummey, Christian, Corben, Louise, Delatycki, Martin, Wilmot, George, Subramony, Sub, Corti, Manuela, Bushara, Khalaf, Duquette, Antoine, Gomez, Christopher, Hoyle, J, Roxburgh, Richard, Seeberger, Lauren, Yoon, Grace, Mathews, Katherine, Zesiewicz, Theresa, Perlman, Susan, and Lynch, David

Subjects
Humans, Friedreich Ataxia, Activities of Daily Living, Clinical Trials as Topic, Walking, Research Design
Abstract

BACKGROUND AND OBJECTIVES: The understanding of the natural history of Friedreich ataxia (FRDA) has improved considerably recently, but patterns of neurologic deterioration are not fully clarified, compromising the assessment of the clinical relevance of effects and guidance for study design. The goal of this study was to acknowledge the broad genetic diversity of the population, especially for younger individuals, and to provide analyses stratified by age to guide population selection in future studies. METHODS: Based on a large natural history study, the FRDA Clinical Outcome Measures study that at the current data cut enrolled 1,115 participants, followed up for 5,287 yearly visits, we present results from the modified FRDA Rating Scale and its subscores. The secondary outcomes included the patient-reported activities of daily living scale, the timed 25-foot walk, and the 9-hole peg test. Long-term progression was modeled using slope analyses within early-onset, typical-onset, intermediate-onset, and late-onset FRDA. To reflect recruitment in clinical trials, short-term changes were analyzed within age-based subpopulations. All analyses were stratified by ambulation status. RESULTS: Long-term progression models stratified by disease severity indicated highly differential disease progression, especially at earlier ages at onset. In the ambulatory phase, decline was driven by axial items assessed by the Upright Stability subscore of the mFARS. The analyses of short-term changes showed slower progression with increasing population age due to decreasing genetic severity. Future clinical studies could reduce population diversity, interpatient variability, and the risk of imbalanced treatment groups by selecting the study population based on the functional capacity (e.g., ambulatory status) and by strict age-based stratification. DISCUSSION: The understanding of the diversity within FRDA populations and their patterns of functional decline provides an essential foundation for future clinical trial design including patient selection and facilitates the interpretation of the clinical relevance of progression detected in FRDA.

Published
2022

7. A Benchmark of Rule-Based and Neural Coreference Resolution in Dutch Novels and News

Author

Poot, Corbèn and van Cranenburgh, Andreas

Subjects
Computer Science - Computation and Language
Abstract

We evaluate a rule-based (Lee et al., 2013) and neural (Lee et al., 2018) coreference system on Dutch datasets of two domains: literary novels and news/Wikipedia text. The results provide insight into the relative strengths of data-driven and knowledge-driven systems, as well as the influence of domain, document length, and annotation schemes. The neural system performs best on news/Wikipedia text, while the rule-based system performs best on literature. The neural system shows weaknesses with limited training data and long documents, while the rule-based system is affected by annotation differences. The code and models used in this paper are available at https://github.com/andreasvc/crac2020, Comment: Accepted for CRAC 2020 @ COLING

Published
2020

8. Systems-level analyses of protein-protein interaction network dysfunctions via epichaperomics identify cancer-specific mechanisms of stress adaptation

Author

Rodina, Anna, Xu, Chao, Digwal, Chander S., Joshi, Suhasini, Patel, Yogita, Santhaseela, Anand R., Bay, Sadik, Merugu, Swathi, Alam, Aftab, Yan, Pengrong, Yang, Chenghua, Roychowdhury, Tanaya, Panchal, Palak, Shrestha, Liza, Kang, Yanlong, Sharma, Sahil, Almodovar, Justina, Corben, Adriana, Alpaugh, Mary L., Modi, Shanu, Guzman, Monica L., Fei, Teng, Taldone, Tony, Ginsberg, Stephen D., Erdjument-Bromage, Hediye, Neubert, Thomas A., Manova-Todorova, Katia, Tsou, Meng-Fu Bryan, Young, Jason C., Wang, Tai, and Chiosis, Gabriela

Published
2023
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9. Scoliosis in Friedreichs ataxia: longitudinal characterization in a large heterogeneous cohort.

Author

Rummey, Christian, Flynn, John, Corben, Louise, Delatycki, Martin, Wilmot, George, Subramony, Sub, Bushara, Khalaf, Duquette, Antoine, Gomez, Christopher, Hoyle, J, Roxburgh, Richard, Seeberger, Lauren, Yoon, Grace, Mathews, Katherine, Zesiewicz, Theresa, Perlman, Susan, and Lynch, David

Subjects
Adolescent, Adult, Age of Onset, Child, Disease Progression, Friedreich Ataxia, Humans, Longitudinal Studies, Middle Aged, Prevalence, Scoliosis, Young Adult
Abstract

OBJECTIVE: The objective of this study was to characterize the incidence and progression of scoliosis in the natural history of Friedreichs ataxia (FRDA) and document the factors leading to the requirement for corrective surgery. METHODS: Data on the prevalence of scoliosis and scoliosis surgery from up to 17 years of follow-up collected during a large natural history study in FRDA (1116 patients at 4928 visits) were summarized descriptively and subjected to time to event analyses. RESULTS: Well over 90% of early or typical FRDA patients (as determined by age of onset) developed intermediate to severe scoliosis, while patients with a later onset (>14 years) had no or much lower prevalence of scoliosis. Diagnosis of scoliosis occurs during the onset of ataxia and in rare cases even prior to that. Major progression follows throughout the growth phase and puberty, leading to the need for surgical intervention in more than 50% of individuals in the most severe subgroup. The youngest patients appear to delay surgery until the end of the growth period, leading to further progression before surgical intervention. Age of onset of FRDA before or after reaching 15 years sharply separated severe and relatively mild incidence and progression of scoliosis. INTERPRETATION: Scoliosis is an important comorbidity of FRDA. Our comprehensive documentation of scoliosis progression in this natural history study provides a baseline for comparison as novel treatments become available.

Published
2021

16. Pathophysiology of SARS-CoV-2: the Mount Sinai COVID-19 autopsy experience

Author

Bryce, Clare, Grimes, Zachary, Pujadas, Elisabet, Ahuja, Sadhna, Beasley, Mary Beth, Albrecht, Randy, Hernandez, Tahyna, Stock, Aryeh, Zhao, Zhen, AlRasheed, Mohamed Rizwan, Chen, Joyce, Li, Li, Wang, Diane, Corben, Adriana, Haines, III, G. Kenneth, Westra, William H., Umphlett, Melissa, Gordon, Ronald E., Reidy, Jason, Petersen, Bruce, Salem, Fadi, Fiel, Maria Isabel, El Jamal, Siraj M., Tsankova, Nadejda M., Houldsworth, Jane, Mussa, Zarmeen, Veremis, Brandon, Sordillo, Emilia, Gitman, Melissa R., Nowak, Michael, Brody, Rachel, Harpaz, Noam, Merad, Miriam, Gnjatic, Sacha, Liu, Wen-Chun, Schotsaert, Michael, Miorin, Lisa, Aydillo Gomez, Teresa A., Ramos-Lopez, Irene, Garcia-Sastre, Adolfo, Donnelly, Ryan, Seigler, Patricia, Keys, Calvin, Cameron, Jennifer, Moultrie, Isaiah, Washington, Kae-Lynn, Treatman, Jacquelyn, Sebra, Robert, Jhang, Jeffrey, Firpo, Adolfo, Lednicky, John, Paniz-Mondolfi, Alberto, Cordon-Cardo, Carlos, and Fowkes, Mary E.

Published
2021
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20. Spontaneously-forming spheroids as an in vitro cancer cell model for anticancer drug screening.

Author

Theodoraki, Maria A, Rezende, Celso O, Chantarasriwong, Oraphin, Corben, Adriana D, Theodorakis, Emmanuel A, and Alpaugh, Mary L

Subjects
Cell Line, Tumor, Spheroids, Cellular, Tumor Cells, Cultured, Animals, Humans, Garcinia, Neoplasms, Xanthones, Antineoplastic Agents, Microscopy, Confocal, Microscopy, Fluorescence, Magnetic Resonance Spectroscopy, Drug Screening Assays, Antitumor, Immunohistochemistry, Drug Evaluation, Preclinical, Neoplasm Transplantation, Inhibitory Concentration 50, Apoptosis, Amino Acid Motifs, breast cancer, drug screening, garcinia xanthone motif, lymphovascular embolus, natural products, Oncology and Carcinogenesis
Abstract

The limited translational value in clinic of analyses performed on 2-D cell cultures has prompted a shift toward the generation of 3-dimensional (3-D) multicellular systems. Here we present a spontaneously-forming in vitro cancer spheroid model, referred to as spheroids(MARY-X), that precisely reflects the pathophysiological features commonly found in tumor tissues and the lymphovascular embolus. In addition, we have developed a rapid, inexpensive means to evaluate response following drug treatment where spheroid dissolution indices from brightfield image analyses are used to construct dose-response curves resulting in relevant IC50 values. Using the spheroids(MARY-X) model, we demonstrate the unique ability of a new class of molecules, containing the caged Garcinia xanthone (CGX) motif, to induce spheroidal dissolution and apoptosis at IC50 values of 0.42 +/-0.02 μM for gambogic acid and 0.66 +/-0.02 μM for MAD28. On the other hand, treatment of spheroids(MARY-X) with various currently approved chemotherapeutics of solid and blood-borne cancer types failed to induce any response as indicated by high dissolution indices and subsequent poor IC50 values, such as 7.8 +/-3.1 μM for paclitaxel. Our studies highlight the significance of the spheroids(MARY-X) model in drug screening and underscore the potential of the CGX motif as a promising anticancer pharmacophore.

Published
2015

21. Pharmacologically controlling protein-protein interactions through epichaperomes for therapeutic vulnerability in cancer

Author

Joshi, Suhasini, Gomes, Erica DaGama, Wang, Tai, Corben, Adriana, Taldone, Tony, Gandu, Srinivasa, Xu, Chao, Sharma, Sahil, Buddaseth, Salma, Yan, Pengrong, Chan, Lon Yin L., Gokce, Askan, Rajasekhar, Vinagolu K., Shrestha, Lisa, Panchal, Palak, Almodovar, Justina, Digwal, Chander S., Rodina, Anna, Merugu, Swathi, Pillarsetty, NagaVaraKishore, Miclea, Vlad, Peter, Radu I., Wang, Wanyan, Ginsberg, Stephen D., Tang, Laura, Mattar, Marissa, de Stanchina, Elisa, Yu, Kenneth H., Lowery, Maeve, Grbovic-Huezo, Olivera, O’Reilly, Eileen M., Janjigian, Yelena, Healey, John H., Jarnagin, William R., Allen, Peter J., Sander, Chris, Erdjument-Bromage, Hediye, Neubert, Thomas A., Leach, Steven D., and Chiosis, Gabriela

Published
2021
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22. Chemical tools for epichaperome-mediated interactome dysfunctions of the central nervous system

Author

Bolaender, Alexander, Zatorska, Danuta, He, Huazhong, Joshi, Suhasini, Sharma, Sahil, Digwal, Chander S., Patel, Hardik J., Sun, Weilin, Imber, Brandon S., Ochiana, Stefan O., Patel, Maulik R., Shrestha, Liza, Shah, Smit. K., Wang, Shuo, Karimov, Rashad, Tao, Hui, Patel, Pallav D., Martin, Ananda Rodilla, Yan, Pengrong, Panchal, Palak, Almodovar, Justina, Corben, Adriana, Rimner, Andreas, Ginsberg, Stephen D., Lyashchenko, Serge, Burnazi, Eva, Ku, Anson, Kalidindi, Teja, Lee, Sang Gyu, Grkovski, Milan, Beattie, Bradley J., Zanzonico, Pat, Lewis, Jason S., Larson, Steve, Rodina, Anna, Pillarsetty, Nagavarakishore, Tabar, Viviane, Dunphy, Mark P., Taldone, Tony, Shimizu, Fumiko, and Chiosis, Gabriela

Published
2021
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24. School Counselors as Supervisors: Examining Qualitative Perspectives of Site-Based Internships

Author

Bloomfield, Corben Paul

Abstract

Experienced school counselors provide site-based supervision to university students during the required internship phase of their graduate training. The site-based school counselor agrees to supervise the intern student and collaborates with the university program to ensure internship requirements are addressed. Unlike other counseling programs requiring additional supervision after graduation, school counselors often begin employment with no additional support. As the responsibilities of school counselors continue to grow and evolve, meaningful supervision with high expectations for supervisees is of increasing importance. Despite the need for adequate supervision, the amount of training school counselors have received in order to provide supervision varies. School counseling internship content and objectives also vary among university programs. Using a qualitative phenomenological design, 12 school counselors with internship supervision experience from across the country were interviewed. Participants were asked to discuss their perceptions of (a) the essential components of school counseling internships, (b) the level of support they have experienced from university programs while providing supervision to graduate student interns, and (c) the amount of supervision training received through the university prior to supervising. Four themes were identified which related to (1) the school counselor-intern student relationship; (2) the school counseling program; (3) direct student services; and (4) the school counselor's interactions with parents, teachers, and community resource providers. Data contained in these themes could serve as recommendations for strengthening school counseling internships. In addition, school counselors' perceived level of university support provided during internships, and the amount of training school counselors received through the university prior to supervising, may provide relevant insights for counselor education programs. [The dissertation citations contained here are published with the permission of ProQuest LLC. Further reproduction is prohibited without permission. Copies of dissertations may be obtained by Telephone (800) 1-800-521-0600. Web page: http://www.proquest.com/en-US/products/dissertations/individuals.shtml.]

Published
2018

30. Interrater Reliability of the Scale for the Assessment and Rating of Ataxia, Berg Balance Scale, and Functional Independence Measure Motor Domain in Individuals With Hereditary Cerebellar Ataxia.

Author

Milne, Sarah C., Roberts, Melissa, Ross, Hannah L., Robinson, Amy, Grove, Kristen, Modderman, Gabrielle, Williams, Shannon, Chua, Jillian, Grootendorst, Alison C., Massey, Libby, Szmulewicz, David J., Delatycki, Martin B., and Corben, Louise A.

Abstract

To determine the interrater reliability of the Scale for the Assessment and Rating of Ataxia (SARA), Berg Balance Scale (BBS), and motor domain of the FIM (m-FIM) administered by physiotherapists in individuals with a hereditary cerebellar ataxia (HCA). Participants were assessed by 1 of 4 physiotherapists. Assessments were video-recorded and the remaining 3 physiotherapists scored the scales for each participant. Raters were blinded to each other's scores. Assessments were administered at 3 clinical locations in separate states in Australia. Twenty-one individuals (mean age=47.63 years; SD=18.42; 13 male and 8 female) living in the community with an HCA were recruited (N=21). Total and single-item scores of the SARA, BBS, and m-FIM were examined. The m-FIM was conducted by interview. Intraclass coefficients (2,1) for the total scores of the m-FIM (0.92; 95% confidence interval [CI], 0.85-0.96), SARA (0.92; 95% CI, 0.86-0.96), and BBS (0.99; 95% CI, 0.98-0.99) indicated excellent interrater reliability. However, there was inconsistent agreement with the individual items, with SARA item 5 (right side) and item 7 (both sides) demonstrating poor interrater reliability and items 1 and 2 demonstrating excellent reliability. The m-FIM (by interview), SARA, and BBS have excellent interrater reliability for use when assessing individuals with an HCA. Physiotherapists could be considered for administration of the SARA in clinical trials. However, further work is required to improve the agreement of the single-item scores and to examine the other psychometric properties of these scales. [ABSTRACT FROM AUTHOR]

Published
2023
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33. Decreased Functional Brain Activation in Friedreich Ataxia Using the Simon Effect Task

Author

Georgiou-Karistianis, N., Akhlaghi, H., and Corben, L. A.

Abstract

The present study applied the Simon effect task to examine the pattern of functional brain reorganization in individuals with Friedreich ataxia (FRDA), using functional magnetic resonance imaging (fMRI). Thirteen individuals with FRDA and 14 age and sex matched controls participated, and were required to respond to either congruent or incongruent arrow stimuli, presented either to the left or right of a screen, via laterally-located button press responses. Although the Simon effect (incongruent minus congruent stimuli) showed common regions of activation in both groups, including the superior and middle prefrontal cortices, insulae, superior and inferior parietal lobules (LPs, LPi), occipital cortex and cerebellum, there was reduced functional activation across a range of brain regions (cortical, subcortical and cerebellar) in individuals with FRDA. The greater Simon effect behaviourally in individuals with FRDA, compared with controls, together with concomitant reductions in functional brain activation and reduced functional connectivity between cortical and sub-cortical regions, implies a likely disruption of cortico-cerebellar loops and ineffective engagement of cognitive/attention regions required for response suppression. (Contains 4 tables and 3 figures.)

Published
2012
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34. Differentiating Profiles of Speech Impairments in Friedreich's Ataxia: A Perceptual and Instrumental Approach

Author

Folker, Joanne E., Murdoch, Bruce E., Rosen, Kristin M., Cahill, Louise M., Delatycki, Martin B., Corben, Louise A., and Vogel, Adam P.

Abstract

Background: The speech disorder associated with Friedreich's ataxia (FRDA) is classically described as ataxic dysarthria. However, variable neuropathology beyond the cerebellum, which may include the corticospinal and corticobulbar tracts, means that the dysarthria can be mixed rather than a pure ataxic dysarthria. Aims: To characterize physiological features of the dysarthria associated with FRDA and identify differential patterns of deviation that may occur across the subsystems of the speech-production mechanism in a series of seven case studies. Methods & Procedures: The assessment battery included a perceptual analysis of a speech sample using an interval rating scale, and a range of instrumental measures to investigate the respiratory, laryngeal, velopharyngeal and articulatory systems. Outcomes & Results: The results demonstrated the variability that exists in the dysarthria associated with FRDA, highlighting the existence of differential profiles of speech impairment. A particular distinction was observed between the presence of hypernasality and phonatory dysfunction, as evidenced by the instrumental results. Conclusions & Implications: The distinct profiles of dysarthria associated with FRDA indicate that approaches that address multiple subsystems are necessary for the accurate characterization and quantification of the motor speech disorder. Further research is required to investigate the decline in speech function as the disease progresses, as changes in speech function over time may be a good indicator of neurological decline in FRDA. (Contains 5 tables.)

Published
2012
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35. Impaired Inhibition of Prepotent Motor Tendencies in Friedreich Ataxia Demonstrated by the Simon Interference Task

Author

Corben, L. A., Akhlaghi, H., and Georgiou-Karistianis, N.

Abstract

Friedreich ataxia (FRDA) is the most common of the genetically inherited ataxias. We recently demonstrated that people with FRDA have impairment in motor planning--most likely because of pathology affecting the cerebral cortex and/or cerebello-cortical projections. We used the Simon interference task to examine how effective 13 individuals with FRDA were at inhibiting inappropriate automatic responses associated with stimulus-response incompatibility in comparison with control participants. Participants had to respond to arrow targets according to two features which were either congruent or incongruent. We found that individuals with FRDA were differentially affected in reaction time to incongruent, compared with congruent stimuli, when compared with control participants. There was a significant negative correlation between age of onset and the incongruency effect, suggesting an impact of FRDA on the developmental unfolding of motor cognition, independent of the effect of disease duration. Future neuroimaging studies will be required to establish whether this dysfunction is due to cerebellar impairment disrupting cerebro-ponto-cerebello-thalamo-cerebral loops (and thus cortical function), direct primary cortical pathology, or a possible combination of the two.

Published
2011
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36. Automatic Method of Pause Measurement for Normal and Dysarthric Speech

Author

Rosen, Kristin, Murdoch, Bruce, Folker, Joanne, Vogel, Adam, Cahill, Louise, Delatycki, Martin, and Corben, Louise

Abstract

This study proposes an automatic method for the detection of pauses and identification of pause types in conversational speech for the purpose of measuring the effects of Friedreich's Ataxia (FRDA) on speech. Speech samples of [approximately] 3 minutes were recorded from 13 speakers with FRDA and 18 healthy controls. Pauses were measured from the intensity contour and fit with bimodal lognormal distributions using the Expectation-Maximization algorithm in Matlab[C]. In the speakers with FRDA, both modes in the pause distributions had significantly larger means, with disproportionately fewer pauses associated with the first mode. From this preliminary study, it is concluded that distributional analysis of pause duration holds promise as a useful method of measuring the effects of FRDA on functional speech. (Contains 3 figures, 2 tables and 4 notes.)

Published
2010
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37. Speech Perception Ability in Individuals with Friedreich Ataxia

Author

Rance, Gary, Fava, Rosanne, Baldock, Heath, Chong, April, Barker, Elizabeth, Corben, Louise, and Delatycki

Abstract

The aim of this study was to investigate auditory pathway function and speech perception ability in individuals with Friedreich ataxia (FRDA). Ten subjects confirmed by genetic testing as being homozygous for a GAA expansion in intron 1 of the FXN gene were included. While each of the subjects demonstrated normal, or near normal sound detection, 3 of the 10 showed electrophysiological evidence of auditory pathway disorder [presenting with the auditory neuropathy/dyssynchrony (AN/AD) result pattern], and 9 of the 10 showed abnormal speech understanding when tested with levels of background noise typical of everyday listening conditions. Information transmission analyses of the speech perception findings for the three FRDA subjects with AN/AD type hearing loss when compared with those of a cohort of individuals with peripheral [sensorineural (SN)] hearing loss, showed a distinct pattern of perceptual disruption. Where the listeners with SN loss confused sounds on the basis of frequency (pitch) differences, the FRDA subjects with AN/AD made errors that reflected an inability to perceive temporal (timing) cues in the speech sounds.

Published
2008
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40. An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels

Author

Yiu, Eppie M., Tai, Geneieve, Peverill, Roger E., Lee, Katherine J., Croft, Kevin D., Mori, Trevor A., Scheiber-Mojdehkar, Barbara, Sturm, Brigitte, Praschberger, Monika, Vogel, Adam P., Rance, Gary, Stephenson, Sarah E. M., Sarsero, Joseph P., Stockley, Creina, Lee, Chung-Yung J., Churchyard, Andrew, Evans-Galea, Marguerite V., Ryan, Monique M., Lockhart, Paul J., Corben, Louise A., and Delatycki, Martin B.

Published
2015
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