Small molecule Y-320 stimulates ribosome biogenesis, protein synthesis, and aminoglycoside-induced premature termination codon readthrough
Hosseini-Farahabadi, Sara, Baradaran-Heravi, Alireza, Zimmerman, Carla, Choi, Kunho, Flibotte, Stephane, Roberge, Michel
Published in PLoS biology (03.05.2021)
Published in PLoS biology (03.05.2021)
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Novel small molecules potentiate premature termination codon readthrough by aminoglycosides
Baradaran-Heravi, Alireza, Balgi, Aruna D, Zimmerman, Carla, Choi, Kunho, Shidmoossavee, Fahimeh S, Tan, Jason S, Bergeaud, Célia, Krause, Alexandra, Flibotte, Stéphane, Shimizu, Yoko, Anderson, Hilary J, Mouly, Vincent, Jan, Eric, Pfeifer, Tom, Jaquith, James B, Roberge, Michel
Published in Nucleic acids research (19.08.2016)
Published in Nucleic acids research (19.08.2016)
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Increased Wnt and Notch signaling: a clue to the renal disease in Schimke immuno-osseous dysplasia?
Morimoto, Marie, Myung, Clara, Beirnes, Kimberly, Choi, Kunho, Asakura, Yumi, Bokenkamp, Arend, Bonneau, Dominique, Brugnara, Milena, Charrow, Joel, Colin, Estelle, Davis, Amira, Deschenes, Georges, Gentile, Mattia, Giordano, Mario, Gormley, Andrew K, Govender, Rajeshree, Joseph, Mark, Keller, Kory, Lerut, Evelyne, Levtchenko, Elena, Massella, Laura, Mayfield, Christy, Najafian, Behzad, Parham, David, Spranger, Jurgen, Stenzel, Peter, Yis, Uluc, Yu, Zhongxin, Zonana, Jonathan, Hendson, Glenda, Boerkoel, Cornelius F
Published in Orphanet journal of rare diseases (05.11.2016)
Published in Orphanet journal of rare diseases (05.11.2016)
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Neurologic Phenotype of Schimke Immuno-Osseous Dysplasia and Neurodevelopmental Expression of SMARCAL1
Deguchi, Kimiko, Clewing, Johanna M, Elizondo, Leah I, Hirano, Ryuki, Huang, Cheng, Choi, Kunho, Sloan, Emily A, Lücke, Thomas, Marwedel, Katja M, Powell, Ralph D, Santa Cruz, Karen, Willaime-Morawek, Sandrine, Inoue, Ken, Lou, Shu, Northrop, Jennifer L, Kanemura, Yonehiro, van der Kooy, Derek, Okano, Hideyuki, Armstrong, Dawna L, Boerkoel, Cornelius F
Published in Journal of neuropathology and experimental neurology (01.06.2008)
Published in Journal of neuropathology and experimental neurology (01.06.2008)
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Wls provides a new compartmental view of the rhombic lip in mouse cerebellar development
Yeung, Joanna, Ha, Thomas J, Swanson, Douglas J, Choi, Kunho, Tong, Yiai, Goldowitz, Dan
Published in The Journal of neuroscience (10.09.2014)
Published in The Journal of neuroscience (10.09.2014)
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Nonselective TRPC channel inhibition and suppression of aminoglycoside-induced premature termination codon readthrough by the small molecule AC1903
Baradaran-Heravi, Alireza, Bauer, Claudia C., Pickles, Isabelle B., Hosseini-Farahabadi, Sara, Balgi, Aruna D., Choi, Kunho, Linley, Deborah M., Beech, David J., Roberge, Michel, Bon, Robin S.
Published in The Journal of biological chemistry (01.02.2022)
Published in The Journal of biological chemistry (01.02.2022)
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Gentamicin B1 is a minor gentamicin component with major nonsense mutation suppression activity
Baradaran-Heravi, Alireza, Niesser, Jürgen, Balgi, Aruna D., Choi, Kunho, Zimmerman, Carla, South, Andrew P., Anderson, Hilary J., Strynadka, Natalie C., Bally, Marcel B., Roberge, Michel
Published in Proceedings of the National Academy of Sciences - PNAS (28.03.2017)
Published in Proceedings of the National Academy of Sciences - PNAS (28.03.2017)
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RETRACTED: Gentamicin B1 is a minor gentamicin component with major nonsense mutation suppression activity
Baradaran-Heravi, Alireza, Niesser, Jürgen, Balgi, Aruna D., Choi, Kunho, Zimmerman, Carla, South, Andrew P., Anderson, Hilary J., Strynadka, Natalie C., Bally, Marcel B., Roberge, Michel
Published in Proceedings of the National Academy of Sciences - PNAS (28.03.2017)
Published in Proceedings of the National Academy of Sciences - PNAS (28.03.2017)
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Journal Article
Spinocerebellar ataxia with axonal neuropathy: consequence of a Tdp1 recessive neomorphic mutation?
Hirano, Ryuki, Interthal, Heidrun, Huang, Cheng, Nakamura, Tomonori, Deguchi, Kimiko, Choi, Kunho, Bhattacharjee, Meenakshi B, Arimura, Kimiyoshi, Umehara, Fujio, Izumo, Shuji, Northrop, Jennifer L, Salih, Mustafa AM, Inoue, Ken, Armstrong, Dawna L, Champoux, James J, Takashima, Hiroshi, Boerkoel, Cornelius F
Published in The EMBO journal (14.11.2007)
Published in The EMBO journal (14.11.2007)
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Penetrance of biallelic SMARCAL1 mutations is associated with environmental and genetic disturbances of gene expression
Baradaran-Heravi, Alireza, Cho, Kyoung Sang, Tolhuis, Bas, Sanyal, Mrinmoy, Morozova, Olena, Morimoto, Marie, Elizondo, Leah I, Bridgewater, Darren, Lubieniecka, Joanna, Beirnes, Kimberly, Myung, Clara, Leung, Danny, Fam, Hok Khim, Choi, Kunho, Huang, Yan, Dionis, Kira Y, Zonana, Jonathan, Keller, Kory, Stenzel, Peter, Mayfield, Christy, Lücke, Thomas, Bokenkamp, Arend, Marra, Marco A, van Lohuizen, Maarten, Lewis, David B, Shaw, Chad, Boerkoel, Cornelius F
Published in Human molecular genetics (01.06.2012)
Published in Human molecular genetics (01.06.2012)
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Identification of a putative Tdp1 inhibitor (CD00509) by in vitro and cell-based assays
Dean, Richard A, Fam, Hok Khim, An, Jianghong, Choi, Kunho, Shimizu, Yoko, Jones, Steven J M, Boerkoel, Cornelius F, Interthal, Heidrun, Pfeifer, Tom A
Published in Journal of biomolecular screening (01.12.2014)
Published in Journal of biomolecular screening (01.12.2014)
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Journal Article
TDP1 and PARP1 deficiency are cytotoxic to rhabdomyosarcoma cells
Fam, Hok Khim, Walton, Cheryl, Mitra, Sheetal A, Chowdhury, Miraj, Osborne, Nichola, Choi, Kunho, Sun, Guobin, Wong, Patrick C W, O'Sullivan, Maureen J, Turashvili, Gulisa, Aparicio, Samuel, Triche, Timothy J, Bond, Mason, Pallen, Catherine J, Boerkoel, Cornelius F
Published in Molecular cancer research (01.10.2013)
Published in Molecular cancer research (01.10.2013)
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Desmosterolosis: an illustration of diagnostic ambiguity of cholesterol synthesis disorders
Dias, Cristina, Rupps, Rosemarie, Millar, Benjamin, Choi, Kunho, Marra, Marco, Demos, Michelle, Kratz, Lisa E, Boerkoel, Cornelius F
Published in Orphanet journal of rare diseases (25.06.2014)
Published in Orphanet journal of rare diseases (25.06.2014)
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Genome-wide microarray comparison reveals downstream genes of Pax6 in the developing mouse cerebellum
Ha, Thomas J., Swanson, Douglas J., Kirova, Roumyana, Yeung, Joanna, Choi, Kunho, Tong, Yiai, Chesler, Elissa J., Goldowitz, Daniel
Published in The European journal of neuroscience (01.10.2012)
Published in The European journal of neuroscience (01.10.2012)
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Journal Article
Lack of IL7Rα expression in T cells is a hallmark of T-cell immunodeficiency in Schimke immuno-osseous dysplasia (SIOD)
Sanyal, Mrinmoy, Morimoto, Marie, Baradaran-Heravi, Alireza, Choi, Kunho, Kambham, Neeraja, Jensen, Kent, Dutt, Suparna, Dionis-Petersen, Kira Y, Liu, Lan Xiang, Felix, Katie, Mayfield, Christy, Dekel, Benjamin, Bokenkamp, Arend, Fryssira, Helen, Guillen-Navarro, Encarna, Lama, Giuliana, Brugnara, Milena, Lücke, Thomas, Olney, Ann Haskins, Hunley, Tracy E, Polat, Ayse Ipek, Yis, Uluc, Bogdanovic, Radovan, Mitrovic, Katarina, Berry, Susan, Najera, Lydia, Najafian, Behzad, Gentile, Mattia, Nur Semerci, C, Tsimaratos, Michel, Lewis, David B, Boerkoel, Cornelius F
Published in Clinical immunology (Orlando, Fla.) (01.12.2015)
Published in Clinical immunology (Orlando, Fla.) (01.12.2015)
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Elucidating the role of mitochondrial Tdp1 in response to oxidative stress
Fam, Hok Khim, Chowdhury, Miraj K., Walton, Cheryl, Choi, Kunho, Hendson, Glenda, Boerkoel, Cornelius F.
Published in Mitochondrion (01.11.2013)
Published in Mitochondrion (01.11.2013)
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