Faster Sensitivity Loss around Dense Scotomas than for Overall Macular Sensitivity in Stargardt Disease: ProgStar Report No. 14

Autor: Etienne M. Schönbach, Rupert W. Strauss, Mohamed A. Ibrahim, Jessica L. Janes, David G. Birch, Artur V. Cideciyan, Janet S. Sunness, Beatriz Muñoz, Michael S. Ip, SriniVas R. Sadda, Hendrik P.N. Scholl, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Burgnoni, Carol Applegate, Leslie Russell, Michel Michaelides, Simona Degli Esposti, Anthony Moore, Andrew Webster, Sophie Connor, Jade Barnfield, Zaid Salchi, Clara Alfageme, Victoria McCudden, Maria Pefkianaki, Jonathan Aboshiha, Gerald Liew, Graham Holder, Anthony Robson, Alexa King, Daniela Ivanova Cajas Narvaez, Katy Barnard, Catherine Grigg, Hannah Dunbar, Yetunde Obadeyi, Karine Girard-Claudon, Hilary Swann, Avani Rughani, Charles Amoah, Dominic Carrington, Kanom Bibi, Emerson Ting, Mohamed Nafaz Illiyas, Hamida Begum, Andrew Carter, Anne Georgiou, Selma Lewism, Saddaf Shaheen, Harpreet Shinmar, Linda Burton, Paul Bernstein, Kimberley Wegner, Briana Lauren Sawyer, Bonnie Carlstrom, Kellian Farnsworth, Cyrie Fry, Melissa Chandler, Glen Jenkins, Donnel Creel, David Birch, Yi-Zhong Wang, Luis Rodriguez, Kirsten Locke, Martin Klein, Paulina Mejia, Samuel G. Jacobson, Sharon B. Schwartz, Rodrigo Matsui, Michaela Gruzensky, Jason Charng, Alejandro J. Roman, Eberhart Zrenner, Fadi Nasser, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, José-Alain Sahel, Saddek Mohand-Said, Isabelle Audo, Caroline Laurent-Coriat, Ieva Sliesoraityte, Christina Zeitz, Fiona Boyard, Minh Ha Tran, Mathias Chapon, Céline Chaumette, Juliette Amaudruz, Victoria Ganem, Serge Sancho, Aurore Girmens, Robert Wojciechowski, Shazia Khan, David G. Emmert, Dennis Cain, Mark Herring, Jennifer Bassinger, Lisa Liberto, Sheila West, Ann-Margret Ervin, Beatriz Munoz, Xiangrong Kong, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Anamika Jha, Alex Ho, Brendan Kramer, Ngoc Lam, Rita Tawdros, Yong Dong Zhou, Johana Carmona, Akihito Uji, Amirhossein Hariri, Amy Lock, Anthony Elshafei, Anushika Ganegoda, Christine Petrossian, Dennis Jenkins, Edward Strnad, Elmira Baghdasaryan, Eric Ito, Feliz Samson, Gloria Blanquel, Handan Akil, Jhanisus Melendez, Jianqin Lei, Jianyan Huang, Jonathan Chau, Khalil G. Falavarjani, Kristina Espino, Manfred Li, Maria Mendoza, Muneeswar Gupta Nittala, Netali Roded, Nizar Saleh, Ping Huang, Sean Pitetta, Siva Balasubramanian, Sophie Leahy, Sowmya J. Srinivas, Swetha B. Velaga, Teresa Margaryan, Tudor Tepelus, Tyler Brown, Wenying Fan, Yamileth Murillo, Yue Shi, Katherine Aguilar, Cynthia Chan, Lisa Santos, Brian Seo, Christopher Sison, Silvia Perez, Stephanie Chao, Kelly Miyasato, Julia Higgins, Zoila Luna, Anita Menchaca, Norma Gonzalez, Vicky Robledo, Karen Carig, Kirstie Baker, David Ellenbogen, Daniel Bluemel, Theo Sanford, Daisy Linares, Mei Tran, Lorane Nava, Michelle Oberoi, Mark Romero, Vivian Chiguil, Grantley Bynum-Bain, Monica Kim, Carolina Mendiguren, Xiwen Huang, Monika Smith, Natalie Sarreal

Publikováno v: American Journal of Ophthalmology. 216:219-225

Mean sensitivity (MS) derived from a standard test grid using microperimetry is a sensitive outcome measure in clinical trials investigating new treatments for degenerative retinal diseases. This study hypothesizes that the functional decline is fast

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::88178b9805bd72b62581e10222d655b7
https://doi.org/10.1016/j.ajo.2020.03.020

Alu complementary DNA is enriched in atrophic macular degeneration and triggers retinal pigmented epithelium toxicity via cytosolic innate immunity

Autor: Shuichiro Hirahara, Tetsuro Oshika, Siddharth Narendran, J. Ignacio Aguirre, Shinichi Fukuda, Meenakshi Ambati, Benjamin J. Fowler, Praveen Yerramothu, Bradley D. Gelfand, David R. Hinton, Jayakrishna Ambati, Gerald G. Schumann, Ulrike Held, Yosuke Nagasaka, Felipe Pereira, Kirstie Baker, Nagaraj Kerur, Srinivas R. Sadda, Sanford H. Feldman, Shaobin Wang, Akhil Varshney, Peirong Huang, Ryan D. Makin, Kameshwari Ambati, Vera L. Bonilha, Mo Wang, Tetsuhiro Yasuma, Reo Yasuma, Vidya L. Ambati, Elmira Baghdasaryan, Xiwen Huang, Hiroko Terasaki, Daipayan Banerjee, Ivana Apicella, Kenneth M. Marion, Genrich V. Tolstonog, Yuichiro Ogura, Deepak Bhattarai, Kyung Bo Kim

Publikováno v: Science advances, 7(40):eabj3658
Science Advances
Science advances, vol. 7, no. 40, pp. eabj3658

Description
Endogenous Alu cDNA triggers inflammasome-mediated cell death in age-related macular degeneration.
Long interspersed nuclear element-1 (L1)–mediated reverse transcription (RT) of Alu RNA into cytoplasmic Alu complementary DNA

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ee76950f2faadbc056f974ac06c1a507
https://doi.org/10.1126/sciadv.abj3658

Nucleoside reverse transcriptase inhibitors and Kamuvudines inhibit amyloid-β induced retinal pigmented epithelium degeneration

Autor: Bradley D. Gelfand, Yosuke Nagasaka, Peirong Huang, Kenneth M. Marion, Shaobin Wang, Jayakrishna Ambati, Vidya L. Ambati, Shinichi Fukuda, Kirstie Baker, Srinivas R. Sadda, Siddharth Narendran, Akhil Varshney, Ivana Apicella, Meenakshi Ambati, Jan M. Deussing, Praveen Yerramothu, Felipe Pereira, Younghee Kim, Kameshwari Ambati, Shuichiro Hirahara

Publikováno v: Signal Transduction and Targeted Therapy
Signal Transduction and Targeted Therapy, Vol 6, Iss 1, Pp 1-9 (2021)

Nonfibrillar amyloid-β oligomers (AβOs) are a major component of drusen, the sub-retinal pigmented epithelium (RPE) extracellular deposits characteristic of age-related macular degeneration (AMD), a common cause of global blindness. We report that

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0603e8968fe6efbce84f844c2cff458f
https://doi.org/10.1038/s41392-021-00537-z

Cytoplasmic synthesis of endogenous Alu complementary DNA via reverse transcription and implications in age-related macular degeneration

Autor: Vidya L. Ambati, Mo Wang, Kyung Bo Kim, Joseph R. Herdy, Siddharth Narendran, Yuichiro Ogura, Bradley D. Gelfand, Eamonn Magner, Jayakrishna Ambati, Apuã C. M. Paquola, Christian Röver, Benjamin J. Fowler, Timothy Hanson, Meenakshi Ambati, Lekha Pandya, Ramendra K. Singh, Yosuke Nagasaka, Kirstie Baker, Jian Sun, James W. Hardin, Jinze Liu, Reo Yasuma, Charles L. Bennett, Genrich V. Tolstonog, Deepak Bhattarai, Praveen Yerramothu, Akshat Pandey, Daipayan Banerjee, Tetsuro Oshika, Gerald G. Schumann, Xinan Liu, Srinivas R. Sadda, Kameshwari Ambati, Tammy H. Cummings, Shaban Darwish, Akhil Varshney, Keykavous Parang, Saghar Mozaffari, Ryan D. Makin, Peirong Huang, Kenneth M. Marion, Elmira Baghdasaryan, Shaobin Wang, Hannah Leung, Brian C. Werner, Jennifer A. Erwin, Nagaraj Kerur, Xiwen Huang, Hiroko Terasaki, Felipe Pereira, S Scott Sutton, Shuichiro Hirahara, David R. Hinton, Ulrike Held, Joseph Magagnoli, Shinichi Fukuda, Ivana Apicella, Fred H. Gage, Tetsuhiro Yasuma

Publikováno v: Proceedings of the National Academy of Sciences. 118

Significance Alu elements, comprising more than 10% of the human genome, propagate via retrotransposition. This genomic expansion requires enzymatic activity of L1 that reverse transcribes Alu RNA into Alu cDNA in the nucleus. We report Alu also unde

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::faab56470358e690f442ac6be149dcb9
https://doi.org/10.1073/pnas.2022751118

Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease: ProgStar Report No. 12

Autor: Etienne M. Schönbach, Rupert W. Strauss, Xiangrong Kong, Beatriz Muñoz, Mohamed A. Ibrahim, Janet S. Sunness, David G. Birch, Gesa-Astrid Hahn, Fadi Nasser, Eberhart Zrenner, SriniVas R. Sadda, Sheila K. West, Hendrik P.N. Scholl, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Burgnoni, Carol Applegate, Leslie Russell, Michel Michaelides, Simona Degli Esposti, Anthony Moore, Andrew Webster, Sophie Connor, Jade Barnfield, Zaid Salchi, Clara Alfageme, Victoria McCudden, Maria Pefkianaki, Jonathan Aboshiha, Gerald Liew, Graham Holder, Anthony Robson, Alexa King, Daniela Ivanova Cajas Narvaez, Katy Barnard, Catherine Grigg, Hannah Dunbar, Yetunde Obadeyi, Karine Girard-Claudon, Hilary Swann, Avani Rughani, Charles Amoah, Dominic Carrington, Kanom Bibi, Emerson Ting Co, Mohamed Nafaz Illiyas, Hamida Begum, Andrew Carter, Anne Georgiou, Selma Lewism, Saddaf Shaheen, Harpreet Shinmar, Linda Burton, Paul Bernstein, Kimberley Wegner, Briana Lauren Sawyer, Bonnie Carlstrom, Kellian Farnsworth, Cyrie Fry, Melissa Chandler, Glen Jenkins, Donnel Creel, David Birch, Yi-Zhong Wang, Luis Rodriguez, Kirsten Locke, Martin Klein, Paulina Mejia, Artur V. Cideciyan, Samuel G. Jacobson, Sharon B. Schwartz, Rodrigo Matsui, Michaela Gruzensky, Jason Charng, Alejandro J. Roman, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, José-Alain Sahel, Saddek Mohand-Said, Isabelle Audo, Caroline Laurent-Coriat, Ieva Sliesoraityte, Christina Zeitz, Fiona Boyard, Minh Ha Tran, Mathias Chapon, Céline Chaumette, Juliette Amaudruz, Victoria Ganem, Serge Sancho, Aurore Girmens, Robert Wojciechowski, Shazia Khan, David G. Emmert, Dennis Cain, Mark Herring, Jennifer Bassinger, Lisa Liberto, Sheila West, Ann-Margret Ervin, Beatriz Munoz, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Michael S. Ip, Anamika Jha, Alex Ho, Brendan Kramer, Ngoc Lam, Rita Tawdros, Yong Dong Zhou, Johana Carmona, Akihito Uji, Amirhossein Hariri, Amy Lock, Anthony Elshafei, Anushika Ganegoda, Christine Petrossian, Dennis Jenkins, Edward Strnad, Elmira Baghdasaryan, Eric Ito, Feliz Samson, Gloria Blanquel, Handan Akil, Jhanisus Melendez, Jianqin Lei, Jianyan Huang, Jonathan Chau, Khalil G. Falavarjani, Kristina Espino, Manfred Li, Maria Mendoza, Muneeswar Gupta Nittala, Netali Roded, Nizar Saleh, Ping Huang, Sean Pitetta, Siva Balasubramanian, Sophie Leahy, Sowmya J. Srinivas, Swetha B. Velaga, Teresa Margaryan, Tudor Tepelus, Tyler Brown, Wenying Fan, Yamileth Murillo, Yue Shi, Katherine Aguilar, Cynthia Chan, Lisa Santos, Brian Seo, Christopher Sison, Silvia Perez, Stephanie Chao, Kelly Miyasato, Julia Higgins, Zoila Luna, Anita Menchaca, Norma Gonzalez, Vicky Robledo, Karen Carig, Kirstie Baker, David Ellenbogen, Daniel Bluemel, Theo Sanford, Daisy Linares, Mei Tran, Lorane Nava, Michelle Oberoi, Mark Romero, Vivian Chiguil, Grantley Bynum-Bain, Monica Kim, Carolina Mendiguren, Xiwen Huang, Monika Smith, Natalie Sarreal

Publikováno v: American Journal of Ophthalmology. 193:54-61

Purpose To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design Multicenter, international, prospective cohort study. Methods Fixation testing was performed using the Nidek MP-1 microperi

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4dc1fe9a946f9b63f1bd9660c98d914d
https://doi.org/10.1016/j.ajo.2018.06.003