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128 results on '"Chae, J. H."'

1. Optical investigations on electronic structure changes related to the metal-insulator transition in VO2 film

Author

Koo, C. H., Lee, J. S., Kim, M. W., Chang, Y. J., Noh, T. W., Chae, J. H. Jung B. G., and Kim, H. -T.

Subjects
Condensed Matter - Strongly Correlated Electrons
Abstract

We investigated optical absorption coefficient spectra of an epitaxial VO2 film in wide photon energy (0.5 - 5.0 eV) and temperature (100 - 380 K) regions. In its insulating phase, we observed two d-d transition peaks around 1.3 eV and 2.7 eV and a charge transfer peak around 4.0 eV. As temperature goes above the metal-insulator transition temperature near 340 K, a large portion of the spectral weight of the peak around 4.0 eV becomes redistributed and a Drude-like peak appears. We initially applied the band picture to explain the details of the spectral weight changes, especially the temperature-dependent shift at 2.7 eV, but failed. To check whether the spectral changes are optical signatures of the electron-electron correlation effects, we applied the Hubbard model which takes into account orbital degeneracy. This orbitally degenerate Hubbard model could explain the details of the temperature-dependent peak shifts quite well. In addition, from the peak assignments based on the orbitally degenerate Hubbard model, we could obtain the values of U + delta(~ 3.4 eV) and JH (~ 0.7 eV), where U, delta, and JH are the on-site Coulomb repulsion energy, the crystal field splitting between the t2g bands, and the Hund's rule exchange energy, respectively. Our spectroscopic studies indicate that the electron-electron correlation could play an important role in the metal-insulator transition of VO2.

Published
2005

10. Towards regulatory endorsement of drug development tools to promote the application of model-informed drug development in Duchenne muscular dystrophy

Author

Conrado, D. J., Larkindale, J., Berg, A., Hill, M., Burton, J., Abrams, K. R., Abresch, R. T., Bronson, A., Chapman, D., Crowther, M., Duong, T., Gordish-Dressman, H., Harnisch, L., Henricson, E., Kim, S., Mcdonald, C. M., Schmidt, S., Vong, C., Wang, X., Wong, B. L., Yong, F., Romero, K., Vishwanathan, V., Chidambaranathan, S., Douglas Biggar, W., Mcadam, L. C., Mah, J. K., Tulinius, M., Cnaan, A., Morgenroth, L. P., Leshner, R., Tesi-Rocha, C., Thangarajh, M., Kornberg, A., Ryan, M., Nevo, Y., Dubrovsky, A., Clemens, P. R., Abdel-Hamid, H., Connolly, A. M., Pestronk, A., Teasley, J., Bertorini, T. E., Webster, R., Kolski, H., Kuntz, N., Driscoll, S., Bodensteiner, J. B., Gorni, K., Lotze, T., Day, J. W., Karachunski, P., Henricson, E. K., Joyce, N. C., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Vilchez, J. J., Voit, T., Wong, B., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., Devaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. H., Jongno-Gu, D. R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Kudr, M., Mathews, K., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Selby, K., Tennekoon, G., Vita, G., Apkon, S., Barohn, R., Belousova, E., Brandsema, J., Bruno, C., Burnette, W., Butterfield, R., Byrne, B., Carlo, J., Chandratre, S., Comi, G., Connolly, A., De Groot I, I., Deconinck, N., Dooley, J., Durigneux, J., Finkel, R., Frank, L. M., Harper, A., Hattori, A., Herguner, O., Iannaccone, S., Janas, J., Jong, Y. J., Komaki, H., Lee, W. T., Leung, E., Mah, J., Mercuri, E., Mcmillan, H., Mueller-Felber, W., de Munain A, L., Nakamura, A., Niks, E., Ogata, K., Pascual, S., Pegoraro, E., Renfroe, B., Sanka, R. B., Schallner, J., Sendra, I. I., Servais, L., Smith, E., Sparks, S., Victor, R., Wicklund, M., Wilichoswki, E., Carter, G. T., and Servais, LJP

Subjects
Orphan Drug Production, Duchenne muscular dystrophy, Pharmacy, Model-informed drug development, 030226 pharmacology & pharmacy, Models, Biological, Food and drug administration, 03 medical and health sciences, 0302 clinical medicine, Models, medicine, Humans, Regulatory science, Computer Simulation, Muscular Dystrophy, Drug development tools, Duchenne muscular dystrophy consortium (D-RSC), Rare diseases, Regulatory endorsement, Clinical Trials as Topic, Muscular Dystrophy, Duchenne, United States, United States Food and Drug Administration, Pharmacology, Protocol (science), business.industry, Duchenne, Biological, medicine.disease, Clinical trial, Risk analysis (engineering), Drug development, 030220 oncology & carcinogenesis, Aggregate data, Business
Abstract

Drug development for rare diseases is challenged by small populations and limited data. This makes development of clinical trial protocols difficult and contributes to the uncertainty around whether or not a potential therapy is efficacious. The use of data standards to aggregate data from multiple sources, and the use of such integrated databases to develop statistical models can inform protocol development and reduce the risks in developing new therapies. Achieving regulatory endorsement of such models through defined pathways at the US Food and Drug Administration and European Medicines Authority allows such tools to be used by the drug development community for defined contexts of use without further need for discussion of the underlying model(s). The Duchenne Regulatory Science Consortium (D-RSC) has brought together multiple stakeholders to develop a clinical trial simulation tool for Duchenne muscular dystrophy using such an approach. Here we describe the work of D-RSC as an example of how such an approach may be effective at reducing uncertainty in drug development for rare diseases, and thus bringing effective therapies to patients faster.

Published
2019

11. AMPA receptor GluA2 subunit defects are a cause of neurodevelopmental disorders

Author

Salpietro, V. Dixon, C.L. Guo, H. Bello, O.D. Vandrovcova, J. Efthymiou, S. Maroofian, R. Heimer, G. Burglen, L. Valence, S. Torti, E. Hacke, M. Rankin, J. Tariq, H. Colin, E. Procaccio, V. Striano, P. Mankad, K. Lieb, A. Chen, S. Pisani, L. Bettencourt, C. Männikkö, R. Manole, A. Brusco, A. Grosso, E. Ferrero, G.B. Armstrong-Moron, J. Gueden, S. Bar-Yosef, O. Tzadok, M. Monaghan, K.G. Santiago-Sim, T. Person, R.E. Cho, M.T. Willaert, R. Yoo, Y. Chae, J.-H. Quan, Y. Wu, H. Wang, T. Bernier, R.A. Xia, K. Blesson, A. Jain, M. Motazacker, M.M. Jaeger, B. Schneider, A.L. Boysen, K. Muir, A.M. Myers, C.T. Gavrilova, R.H. Gunderson, L. Schultz-Rogers, L. Klee, E.W. Dyment, D. Osmond, M. Parellada, M. Llorente, C. Gonzalez-Peñas, J. Carracedo, A. Van Haeringen, A. Ruivenkamp, C. Nava, C. Heron, D. Nardello, R. Iacomino, M. Minetti, C. Skabar, A. Fabretto, A. Hanna, M.G. Bugiardini, E. Hostettler, I. O’Callaghan, B. Khan, A. Cortese, A. O’Connor, E. Yau, W.Y. Bourinaris, T. Kaiyrzhanov, R. Chelban, V. Madej, M. Diana, M.C. Vari, M.S. Pedemonte, M. Bruno, C. Balagura, G. Scala, M. Fiorillo, C. Nobili, L. Malintan, N.T. Zanetti, M.N. Krishnakumar, S.S. Lignani, G. Jepson, J.E.C. Broda, P. Baldassari, S. Rossi, P. Fruscione, F. Madia, F. Traverso, M. De-Marco, P. Pérez-Dueñas, B. Munell, F. Kriouile, Y. El-Khorassani, M. Karashova, B. Avdjieva, D. Kathom, H. Tincheva, R. Van-Maldergem, L. Nachbauer, W. Boesch, S. Gagliano, A. Amadori, E. Goraya, J.S. Sultan, T. Kirmani, S. Ibrahim, S. Jan, F. Mine, J. Banu, S. Veggiotti, P. Zuccotti, G.V. Ferrari, M.D. Van Den Maagdenberg, A.M.J. Verrotti, A. Marseglia, G.L. Savasta, S. Soler, M.A. Scuderi, C. Borgione, E. Chimenz, R. Gitto, E. Dipasquale, V. Sallemi, A. Fusco, M. Cuppari, C. Cutrupi, M.C. Ruggieri, M. Cama, A. Capra, V. Mencacci, N.E. Boles, R. Gupta, N. Kabra, M. Papacostas, S. Zamba-Papanicolaou, E. Dardiotis, E. Maqbool, S. Rana, N. Atawneh, O. Lim, S.Y. Shaikh, F. Koutsis, G. Breza, M. Coviello, D.A. Dauvilliers, Y.A. AlKhawaja, I. AlKhawaja, M. Al-Mutairi, F. Stojkovic, T. Ferrucci, V. Zollo, M. Alkuraya, F.S. Kinali, M. Sherifa, H. Benrhouma, H. Turki, I.B.Y. Tazir, M. Obeid, M. Bakhtadze, S. Saadi, N.W. Zaki, M.S. Triki, C.C. Benfenati, F. Gustincich, S. Kara, M. Belcastro, V. Specchio, N. Capovilla, G. Karimiani, E.G. Salih, A.M. Okubadejo, N.U. Ojo, O.O. Oshinaike, O.O. Oguntunde, O. Wahab, K. Bello, A.H. Abubakar, S. Obiabo, Y. Nwazor, E. Ekenze, O. Williams, U. Iyagba, A. Taiwo, L. Komolafe, M. Senkevich, K. Shashkin, C. Zharkynbekova, N. Koneyev, K. Manizha, G. Isrofilov, M. Guliyeva, U. Salayev, K. Khachatryan, S. Rossi, S. Silvestri, G. Haridy, N. Ramenghi, L.A. Xiromerisiou, G. David, E. Aguennouz, M. Fidani, L. Spanaki, C. Tucci, A. Raspall-Chaure, M. Chez, M. Tsai, A. Fassi, E. Shinawi, M. Constantino, J.N. De Zorzi, R. Fortuna, S. Kok, F. Keren, B. Bonneau, D. Choi, M. Benzeev, B. Zara, F. Mefford, H.C. Scheffer, I.E. Clayton-Smith, J. Macaya, A. Rothman, J.E. Eichler, E.E. Kullmann, D.M. Houlden, H. SYNAPS Study Group

Abstract

AMPA receptors (AMPARs) are tetrameric ligand-gated channels made up of combinations of GluA1-4 subunits encoded by GRIA1-4 genes. GluA2 has an especially important role because, following post-transcriptional editing at the Q607 site, it renders heteromultimeric AMPARs Ca2+-impermeable, with a linear relationship between current and trans-membrane voltage. Here, we report heterozygous de novo GRIA2 mutations in 28 unrelated patients with intellectual disability (ID) and neurodevelopmental abnormalities including autism spectrum disorder (ASD), Rett syndrome-like features, and seizures or developmental epileptic encephalopathy (DEE). In functional expression studies, mutations lead to a decrease in agonist-evoked current mediated by mutant subunits compared to wild-type channels. When GluA2 subunits are co-expressed with GluA1, most GRIA2 mutations cause a decreased current amplitude and some also affect voltage rectification. Our results show that de-novo variants in GRIA2 can cause neurodevelopmental disorders, complementing evidence that other genetic causes of ID, ASD and DEE also disrupt glutamatergic synaptic transmission. © 2019, The Author(s).

Published
2019

12. AMPA receptor GluA2 subunit defects are a cause of neurodevelopmental disorders

Author

Salpietro, V, Dixon, CL, Guo, H, Bello, OD, Vandrovcova, J, Efthymiou, S, Maroofian, R, Heimer, G, Burglen, L, Valence, S, Torti, E, Hacke, M, Rankin, J, Tariq, H, Colin, E, Procaccio, V, Striano, P, Mankad, K, Lieb, A, Chen, S, Pisani, L, Bettencourt, C, Mannikko, R, Manole, A, Brusco, A, Grosso, E, Ferrero, GB, Armstrong-Moron, J, Gueden, S, Bar-Yosef, O, Tzadok, M, Monaghan, KG, Santiago-Sim, T, Person, RE, Cho, MT, Willaert, R, Yoo, Y, Chae, J-H, Quan, Y, Wu, H, Wang, T, Bernier, RA, Xia, K, Blesson, A, Jain, M, Motazacker, MM, Jaeger, B, Schneider, AL, Boysen, K, Muir, AM, Myers, CT, Gavrilova, RH, Gunderson, L, Schultz-Rogers, L, Klee, EW, Dyment, D, Osmond, M, Parellada, M, Llorente, C, Gonzalez-Penas, J, Carracedo, A, Van Haeringen, A, Ruivenkamp, C, Nava, C, Heron, D, Nardello, R, Iacomino, M, Minetti, C, Skabar, A, Fabretto, A, Chez, M, Tsai, A, Fassi, E, Shinawi, M, Constantino, JN, De Zorzi, R, Fortuna, S, Kok, F, Keren, B, Bonneau, D, Choi, M, Benzeev, B, Zara, F, Mefford, HC, Scheffer, IE, Clayton-Smith, J, Macaya, A, Rothman, JE, Eichler, EE, Kullmann, DM, Houlden, H, Raspall-Chaure, M, Hanna, MG, Bugiardini, E, Hostettler, I, O'Callaghan, B, Khan, A, Cortese, A, O'Connor, E, Yau, WY, Bourinaris, T, Kaiyrzhanov, R, Chelban, V, Madej, M, Diana, MC, Vari, MS, Pedemonte, M, Bruno, C, Balagura, G, Scala, M, Fiorillo, C, Nobili, L, Malintan, NT, Zanetti, MN, Krishnakumar, SS, Lignani, G, Jepson, JEC, Broda, P, Baldassari, S, Rossi, P, Fruscione, F, Madia, F, Traverso, M, De-Marco, P, Perez-Duenas, B, Munell, F, Kriouile, Y, El-Khorassani, M, Karashova, B, Avdjieva, D, Kathom, H, Tincheva, R, Van-Maldergem, L, Nachbauer, W, Boesch, S, Gagliano, A, Amadori, E, Goraya, JS, Sultan, T, Kirmani, S, Ibrahim, S, Jan, F, Mine, J, Banu, S, Veggiotti, P, Zuccotti, G, Ferrari, MD, Van Den Maagdenberg, AMJ, Verrotti, A, Marseglia, GL, Savasta, S, Soler, MA, Scuderi, C, Borgione, E, Chimenz, R, Gitto, E, Dipasquale, V, Sallemi, A, Fusco, M, Cuppari, C, Cutrupi, MC, Ruggieri, M, Cama, A, Capra, V, Mencacci, NE, Boles, R, Gupta, N, Kabra, M, Papacostas, S, Zamba-Papanicolaou, E, Dardiotis, E, Maqbool, S, Rana, N, Atawneh, O, Lim, SY, Shaikh, F, Koutsis, G, Breza, M, Coviello, DA, Dauvilliers, YA, AlKhawaja, I, AlKhawaja, M, Al-Mutairi, F, Stojkovic, T, Ferrucci, V, Zollo, M, Alkuraya, FS, Kinali, M, Sherifa, H, Benrhouma, H, Turki, IBY, Tazir, M, Obeid, M, Bakhtadze, S, Saadi, NW, Zaki, MS, Triki, CC, Benfenati, F, Gustincich, S, Kara, M, Belcastro, V, Specchio, N, Capovilla, G, Karimiani, EG, Salih, AM, Okubadejo, NU, Ojo, OO, Oshinaike, OO, Oguntunde, O, Wahab, K, Bello, AH, Abubakar, S, Obiabo, Y, Nwazor, E, Ekenze, O, Williams, U, Iyagba, A, Taiwo, L, Komolafe, M, Senkevich, K, Shashkin, C, Zharkynbekova, N, Koneyev, K, Manizha, G, Isrofilov, M, Guliyeva, U, Salayev, K, Khachatryan, S, Rossi, S, Silvestri, G, Haridy, N, Ramenghi, LA, Xiromerisiou, G, David, E, Aguennouz, M, Fidani, L, Spanaki, C, Tucci, A, Salpietro, V, Dixon, CL, Guo, H, Bello, OD, Vandrovcova, J, Efthymiou, S, Maroofian, R, Heimer, G, Burglen, L, Valence, S, Torti, E, Hacke, M, Rankin, J, Tariq, H, Colin, E, Procaccio, V, Striano, P, Mankad, K, Lieb, A, Chen, S, Pisani, L, Bettencourt, C, Mannikko, R, Manole, A, Brusco, A, Grosso, E, Ferrero, GB, Armstrong-Moron, J, Gueden, S, Bar-Yosef, O, Tzadok, M, Monaghan, KG, Santiago-Sim, T, Person, RE, Cho, MT, Willaert, R, Yoo, Y, Chae, J-H, Quan, Y, Wu, H, Wang, T, Bernier, RA, Xia, K, Blesson, A, Jain, M, Motazacker, MM, Jaeger, B, Schneider, AL, Boysen, K, Muir, AM, Myers, CT, Gavrilova, RH, Gunderson, L, Schultz-Rogers, L, Klee, EW, Dyment, D, Osmond, M, Parellada, M, Llorente, C, Gonzalez-Penas, J, Carracedo, A, Van Haeringen, A, Ruivenkamp, C, Nava, C, Heron, D, Nardello, R, Iacomino, M, Minetti, C, Skabar, A, Fabretto, A, Chez, M, Tsai, A, Fassi, E, Shinawi, M, Constantino, JN, De Zorzi, R, Fortuna, S, Kok, F, Keren, B, Bonneau, D, Choi, M, Benzeev, B, Zara, F, Mefford, HC, Scheffer, IE, Clayton-Smith, J, Macaya, A, Rothman, JE, Eichler, EE, Kullmann, DM, Houlden, H, Raspall-Chaure, M, Hanna, MG, Bugiardini, E, Hostettler, I, O'Callaghan, B, Khan, A, Cortese, A, O'Connor, E, Yau, WY, Bourinaris, T, Kaiyrzhanov, R, Chelban, V, Madej, M, Diana, MC, Vari, MS, Pedemonte, M, Bruno, C, Balagura, G, Scala, M, Fiorillo, C, Nobili, L, Malintan, NT, Zanetti, MN, Krishnakumar, SS, Lignani, G, Jepson, JEC, Broda, P, Baldassari, S, Rossi, P, Fruscione, F, Madia, F, Traverso, M, De-Marco, P, Perez-Duenas, B, Munell, F, Kriouile, Y, El-Khorassani, M, Karashova, B, Avdjieva, D, Kathom, H, Tincheva, R, Van-Maldergem, L, Nachbauer, W, Boesch, S, Gagliano, A, Amadori, E, Goraya, JS, Sultan, T, Kirmani, S, Ibrahim, S, Jan, F, Mine, J, Banu, S, Veggiotti, P, Zuccotti, G, Ferrari, MD, Van Den Maagdenberg, AMJ, Verrotti, A, Marseglia, GL, Savasta, S, Soler, MA, Scuderi, C, Borgione, E, Chimenz, R, Gitto, E, Dipasquale, V, Sallemi, A, Fusco, M, Cuppari, C, Cutrupi, MC, Ruggieri, M, Cama, A, Capra, V, Mencacci, NE, Boles, R, Gupta, N, Kabra, M, Papacostas, S, Zamba-Papanicolaou, E, Dardiotis, E, Maqbool, S, Rana, N, Atawneh, O, Lim, SY, Shaikh, F, Koutsis, G, Breza, M, Coviello, DA, Dauvilliers, YA, AlKhawaja, I, AlKhawaja, M, Al-Mutairi, F, Stojkovic, T, Ferrucci, V, Zollo, M, Alkuraya, FS, Kinali, M, Sherifa, H, Benrhouma, H, Turki, IBY, Tazir, M, Obeid, M, Bakhtadze, S, Saadi, NW, Zaki, MS, Triki, CC, Benfenati, F, Gustincich, S, Kara, M, Belcastro, V, Specchio, N, Capovilla, G, Karimiani, EG, Salih, AM, Okubadejo, NU, Ojo, OO, Oshinaike, OO, Oguntunde, O, Wahab, K, Bello, AH, Abubakar, S, Obiabo, Y, Nwazor, E, Ekenze, O, Williams, U, Iyagba, A, Taiwo, L, Komolafe, M, Senkevich, K, Shashkin, C, Zharkynbekova, N, Koneyev, K, Manizha, G, Isrofilov, M, Guliyeva, U, Salayev, K, Khachatryan, S, Rossi, S, Silvestri, G, Haridy, N, Ramenghi, LA, Xiromerisiou, G, David, E, Aguennouz, M, Fidani, L, Spanaki, C, and Tucci, A

Abstract

AMPA receptors (AMPARs) are tetrameric ligand-gated channels made up of combinations of GluA1-4 subunits encoded by GRIA1-4 genes. GluA2 has an especially important role because, following post-transcriptional editing at the Q607 site, it renders heteromultimeric AMPARs Ca2+-impermeable, with a linear relationship between current and trans-membrane voltage. Here, we report heterozygous de novo GRIA2 mutations in 28 unrelated patients with intellectual disability (ID) and neurodevelopmental abnormalities including autism spectrum disorder (ASD), Rett syndrome-like features, and seizures or developmental epileptic encephalopathy (DEE). In functional expression studies, mutations lead to a decrease in agonist-evoked current mediated by mutant subunits compared to wild-type channels. When GluA2 subunits are co-expressed with GluA1, most GRIA2 mutations cause a decreased current amplitude and some also affect voltage rectification. Our results show that de-novo variants in GRIA2 can cause neurodevelopmental disorders, complementing evidence that other genetic causes of ID, ASD and DEE also disrupt glutamatergic synaptic transmission.

Published
2019

14. A randomized placebo-controlled phase 3 trial of an antisense oligonucleotide, drisapersen, in Duchenne muscular dystrophy

Author

Goemans, Nathalie, primary, Mercuri, Eugenio, additional, Belousova, Elena, additional, Komaki, Hirofumi, additional, Dubrovsky, Alberto, additional, McDonald, Craig M., additional, Kraus, John E., additional, Lourbakos, Afrodite, additional, Lin, Zhengning, additional, Campion, Giles, additional, Wang, Susanne X., additional, Campbell, Craig, additional, Araujo, A., additional, Bertini, E., additional, Born, P., additional, Cances, C., additional, Chabrol, B., additional, Chae, J.-H., additional, Colomer Oferil, J., additional, Comi, G.P., additional, Cuisset, J.-M., additional, D'Anjou, G., additional, Desguerre, I., additional, Erazo Torricelli, R., additional, Escobar, R., additional, Feder, D., additional, Ferlini, A., additional, Giugliani, R., additional, Henricson, E., additional, Herczegfalvi, A., additional, Jong, Y.-J., additional, Kimura, S., additional, Kirschner, J.-B., additional, Kleinsteuber, K., additional, Kostera-Pruszczyk, A., additional, Kudr, M., additional, Mueller-Felber, W., additional, Niks, E.H., additional, Ogata, K., additional, Palermo, C., additional, Pane, M., additional, Pascual, I., additional, Pereon, Y., additional, Raskin, S., additional, Rasmussen, M., additional, Reed, U., additional, Schara, U., additional, Selby, K., additional, Sobreira, C., additional, Takeshima, Y., additional, Vilchez Padilla, J.J., additional, Vita, G., additional, Vondracek, P., additional, Wiegand, G., additional, and Wilichowski, E., additional

Published
2018
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15. Ataluren in patients with nonsense mutation Duchenne muscular dystrophy (ACT DMD): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial

Author

McDonald, C. M., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Tulinius, M., Vilchez, J. J., Voit, T., Wong, B., Elfring, G., Kroger, H., Luo, X., McIntosh, J., Ong, T., Riebling, P., Souza, M., Spiegel, R. J., Peltz, S. W., Mercuri, E., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., de Vaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. -H., Jongno-gu, D. -R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Karachunski, P., Kudr, M., Lotze, T., Mah, J. K., Mathews, K., Nevo, Y., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Ryan, M., Selby, K., Tennekoon, G., Vita, G., Mercuri E. (ORCID:0000-0002-9851-5365), McDonald, C. M., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Tulinius, M., Vilchez, J. J., Voit, T., Wong, B., Elfring, G., Kroger, H., Luo, X., McIntosh, J., Ong, T., Riebling, P., Souza, M., Spiegel, R. J., Peltz, S. W., Mercuri, E., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., de Vaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. -H., Jongno-gu, D. -R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Karachunski, P., Kudr, M., Lotze, T., Mah, J. K., Mathews, K., Nevo, Y., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Ryan, M., Selby, K., Tennekoon, G., Vita, G., and Mercuri E. (ORCID:0000-0002-9851-5365)

Abstract

Background Duchenne muscular dystrophy (DMD) is a severe, progressive, and rare neuromuscular, X-linked recessive disease. Dystrophin deficiency is the underlying cause of disease; therefore, mutation-specific therapies aimed at restoring dystrophin protein production are being explored. We aimed to assess the efficacy and safety of ataluren in ambulatory boys with nonsense mutation DMD. Methods We did this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial at 54 sites in 18 countries located in North America, Europe, the Asia-Pacific region, and Latin America. Boys aged 7–16 years with nonsense mutation DMD and a baseline 6-minute walk distance (6MWD) of 150 m or more and 80% or less of the predicted normal value for age and height were randomly assigned (1:1), via permuted block randomisation (block size of four) using an interactive voice-response or web-response system, to receive ataluren orally three times daily (40 mg/kg per day) or matching placebo. Randomisation was stratified by age (<9 years vs ≥9 years), duration of previous corticosteroid use (6 months to <12 months vs ≥12 months), and baseline 6MWD (<350 m vs ≥350 m). Patients, parents and caregivers, investigational site personnel, PTC Therapeutics employees, and all other study personnel were masked to group allocation until after database lock. The primary endpoint was change in 6MWD from baseline to week 48. We additionally did a prespecified subgroup analysis of the primary endpoint, based on baseline 6MWD, which is reflective of anticipated rates of disease progression over 1 year. The primary analysis was by intention to treat. This study is registered with ClinicalTrials.gov, number NCT01826487. Findings Between March 26, 2013, and Aug 26, 2014, we randomly assigned 230 patients to receive ataluren (n=115) or placebo (n=115); 228 patients comprised the intention-to-treat population. The least-squares mean change in 6MWD from baseline to week 48 was −47·7 m (SE 9·3

Published
2017

18. Age-specific association between body mass index and depression: The Korea National Health and Nutrition Examination Survey 2014

Author

Oh, J, Chae, J-H, and Kim, T-S

Abstract

Background/Objectives:Decades of research have investigated the association between body mass index (BMI) and depression. Although it has been suggested that obesity lowers the risk of depression in elderly adults, the association of overweight and obesity with depression across age groups remains controversial. Thus, we aimed to investigate how the odds of depression vary between BMI groups in the general population.Subjects/Methods:In this study, data were collected from the sixth Korea National Health and Nutrition Examination Survey of 2014 (KNHANES VI; n=7550), and 4932 adults were included in the study sample. We investigated the adjusted association between depression, which was measured by the Patient Health Questionnaire 9 (PHQ-9), and BMI groups (underweight, normal weight, overweight, obese class I, obese classes II and III).Results:In all adults, after adjusting for sex, age, income and presence of chronic illnesses, overweight had lower odds of depression than normal weight (OR, 0.811; 95% CI, 0.661–0.995), whereas underweight had higher odds of depression than normal-weight subjects (OR, 1.776; 95% CI, 1.241–2.540). Overweight elderly adults had lower odds of depression than normal-weight elderly adults (OR, 0.522, 95% CI, 0.367–0.743), and obese class I elderly adults also had lower odds of depression than normal-weight elderly adults in both men (OR, 0.482, 95% CI, 0.251–0.924) and women (OR, 0.637, 95% CI, 0.418–0.970). Furthermore, in the normal-weight-to-obese class I elderly adults group, we found that an increase of 1?kg?m-2BMI was significantly associated with a decreased prevalence of depression in both men (OR, 0.898, 95% CI, 0.808–0.997) and women (OR, 0.911, 95% CI, 0.844–0.982).Conclusions:We found that overweight and mild obesity significantly lowered the risk of depression in elderly adults. These findings suggest that mild increases in BMI beyond the normal range may be a protective factor for depression in elderly Korean adults.

Published
2018
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35. Creatine kinase isoenzyme activity during and after an ultra-distance (200 km) run.

Author

Son, H. J., Lee, Y. H., Chae, J. H., and Kim, C. K.

Abstract

It is commonly assumed that creatine kinase (CK) activity in plasma is related to the state of an inflammatory response at 24-48 h, and also it has shown biphasic patterns after a marathon run. No information is available on CK isoenzymes after an ultra-marathon run. The purpose of the present study is to examine the CK isoenzymes after a 200 km ultra-marathon run and during the subsequent recovery. Blood samples were obtained during registration 1 2 h before the 200-km race and during the race at 100 km, 150 km and at the end of 200 km, as well as after a 24 h period of recovery. Thirty-two male ultra-distance runners participated in the study. Serum CPK showed a marked increase throughout the race and 24 h recovery period (p<0.001). Serum CK during the race occurs mostly in the CK-MM isoform and only minutely in the CK-MB isoform and is unchanged in the CK-BB isoform. High-sensitivity C-reactive protein (hs-CRP), oestradiol, AST and ALT increased significantly from the pre-race value at 100 km and a further increase took place by the end of the 200 km run. The results of our study demonstrate a different release pattern of creatine kinase after an ultra-distance (200 km) run compared to the studies of marathon running and intense eccentric exercise, and changes in several biomarkers, indicative of muscle damage during the race, were much more pronounced during the latter half (100-200 km) of the race. However, the increases in plasma concentration of muscle enzymes may reflect not only structural damage, but also their rate of clearance. [ABSTRACT FROM AUTHOR]

Published
2015
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37. Recent progress of high-power InGaN blue-violet laser diodes

Author

Ryu, H. Y., primary, Ha, K. H., additional, Lee, S. N., additional, Choi, K. K., additional, Jang, T., additional, Son, J. K., additional, Kim, H. G., additional, Chae, J. H., additional, Paek, H. S., additional, Sung, Y. J., additional, Sakong, T., additional, Kim, K. S., additional, Nam, O. H., additional, and Park, Y. J., additional

Published
2006
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38. Highly stable temperature characteristics of InGaN blue laser diodes

Author

Ryu, H. Y., primary, Ha, K. H., additional, Lee, S. N., additional, Jang, T., additional, Kim, H. K., additional, Chae, J. H., additional, Kim, K. S., additional, Choi, K. K., additional, Son, J. K., additional, Paek, H. S., additional, Sung, Y. J., additional, Sakong, T., additional, Nam, O. H., additional, and Park, Y. J., additional

Published
2006
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39. Characteristics of GaN-based laser diodes for post-DVD applications

Author

Nam, O. H., primary, Ha, K. H., additional, Kwak, J. S., additional, Lee, S. N., additional, Choi, K. K., additional, Chang, T. H., additional, Chae, S. H., additional, Lee, W. S., additional, Sung, Y. J., additional, Paek, H. S., additional, Chae, J. H., additional, Sakong, T., additional, Son, J. K., additional, Ryu, H. Y., additional, Kim, Y. H., additional, and Park, Y., additional

Published
2004
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40. Recent progress of high power GaN‐based violet laser diodes

Author

Nam, O. H., primary, Ha, K. H., additional, Kwak, J. S., additional, Lee, S. N., additional, Choi, K. K., additional, Chang, T. H., additional, Chae, S. H., additional, Lee, W. S., additional, Sung, Y. J., additional, Paek, H. S., additional, Chae, J. H., additional, Sakong, T., additional, and Park, Y., additional

Published
2003
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41. Vagus nerve stimulation therapy

Author

George, M.S., primary, Nahas, Z., additional, Bohning, D.E., additional, Kozel, F.A., additional, Anderson, B., additional, Chae, J.-H., additional, Lomarev, M., additional, Denslow, S., additional, Li, X., additional, and Mu, C., additional

Published
2002
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44. A-train CALIOP and MLS observations of early winter antarctic polar stratospheric clouds and nitric acid in 2008.

Author

Lambert, A., Santee, M. L., Wu, D. L., and Chae, J. H.

Abstract

A-train Cloud-Aerosol Lidar with Orthogonal Polarization (CALIOP) and Microwave Limb Sounder (MLS) observations are used to investigate the development of polar stratospheric clouds (PSCs) and the gas phase nitric acid distribution in the early 2008 Antarctic winter. Observational evidence of gravity-wave activity is provided by Atmospheric Infrared Sounder (AIRS) radiances and infrared spectroscopic detection of nitric acid trihydrate (NAT) in PSCs is obtained from the Michelson Interferometer for Passive Atmospheric Sounding (MIPAS). Goddard Earth Observing System Data Assimilation System (GEOS-5 DAS) analyses are used to derive Lagrangian trajectories and to determine temperature-time histories of air parcels. We use CALIOP backscatter and depolarization measurements to classify PSCs and the MLS measurements to determine the corresponding gas phase HNO3 as a function of temperature. For liquid PSCs the uptake of HNO3 follows the theoretical equilibrium curve for supercooled ternary solutions (STS), but at temperatures about 1 K lower as determined from GEOS-5. In the presence of solid phase PSCs, above the ice frost-point, the HNO3 depletion occurs over a wider range of temperatures (+2 to -7 K) distributed about the NAT equilibrium curve. Rapid gas phase HNO3 depletion is first seen by MLS from from 23-25 May 2008, consisting of a decrease in the volume mixing ratio (parts per billion by volume) from 14 ppbv to 7 ppbv on the 46--32 hPa (hectopascal) pressure levels and accompanied by a 2--3 ppbv increase by renitrification at the 68 hPa pressure level. Temperature-time histories of air parcels demonstrate that the depleted HNO3 region is more clearly correlated with prior low temperature exposure of a few kelvin above the frost-point than with either the region bounded by the NAT existence temperature threshold or the region of minimum temperatures. From the combined data we infer the presence of large-size NAT particles with effective radii > 5--7 µm and low NAT number densities < 1 x 10-3 cm-3. This denitrification event is observed close to the pole in the Antarctic vortex before synoptic temperatures first fall below the ice frost point and before the widespread occurrence of large-scale NAT PSCs at altitudes 18--26 km in a polar freezing belt. The NAT outbreak is similar to an event previously reported from MIPAS observations in mid-June 2003 and is again linked to NAT formation via ice-seeding following an episode of mountain wave activity detected by AIRS. Subsequent wave-ice formation in the rapid cooling phases over the Antarctic Peninsula and Ellsworth Mountains is detected here by CALIOP and MIPAS. The NAT clouds appear to be composed of relatively small particles with estimated effective radii of around 1 µm and high NAT number densities >0.2 cm-3. [ABSTRACT FROM AUTHOR]

Published
2011
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45. Characteristics of CALIOP attenuated backscatter noise: implication for cloud/aerosol detection.

Author

Wu, D. L., Chae, J. H., Lambert, A., and Zhang, F. F.

Abstract

To study cloud/aerosol features in the upper troposphere and lower stratosphere (UT/LS) with the NASA's A-Train sensors, a research algorithm is developed for a re-gridded CALIOP (Cloud-Aerosol Lidar with Orthogonal Polarization) Level 1 (L1) backscatter dataset. This paper provides a detailed analysis of the measurement noise of this re-gridded dataset in order to compare the lidar measurements with other collocated measurements (e.g., CloudSat, Microwave Limb Sounder). The regridded dataset has a manageable data volume for multi-year analysis. It has a fixed (5 km) horizontal resolution, and the measurement error is derived empirically from the background-corrected backscatter profile on a profile-by-profile basis. The 532-nm and 1064-nm measurement noises, determined from the data at altitudes above 19 km, are analyzed and characterized in terms of the mean (μ), standard deviation (σ), and normalized probability density function (PDF). These noises show a larger variance over landmasses and bright surfaces during day, and in regions with enhanced flux of energetic particles during night, where the instrument's ability for feature detection is slightly degraded. An increasing trend in the nighttime 1064-nm σ appears to be significant, which likely causes the increasing differences in cloud occurrence frequency between the 532-nm and 1064-nm channels. Most of the CALIOP backscatter noise distributions exhibit a Gaussian-like behavior but the nighttime 532-nm perpendicular measurements show multi-Gaussian characteristics. We apply σ - based thresholds to detect cloud/aerosol features in the UT/LS from the subset L1 data. The observed morphology is similar to that from the Level 2 (L2) 05km CLAY+05km ALAY product, but the occurrence frequency obtained in this study is slightly lower than the L2 product due to differences in spatial averaging and detection threshold. In the case where the measurement noises of two data sets are different, the normalized PDF has proven useful for quantifying the day-night difference of the CALIOP backscatters, showing higher daytime cloud occurrence frequency in the tropical UT/LS. Other cloud/aerosol properties, such as depolarization ratio and color ratio, can be also evaluated with the PDF method. [ABSTRACT FROM AUTHOR]

Published
2010
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46. The role of tropical deep convective clouds on temperature, water vapor, and dehydration in the tropical tropopause layer (TTL).

Author

Chae, J. H., Wu, D. L., Read, W. G., and Sherwood, S. C.

Abstract

Temperature and water vapor variations due to clouds in the TTL have been investigated using co-located MLS, CALISPO, and CloudSat data. Convective cooling occurs only up to cloud top heights, but there is warming above these heights in the TTL. Water vapor and ozone anomalies above cloud top heights support that the warming anomalies occur due to downward motion. Thicker clouds cause a greater magnitude of the temperature anomalies. Water vapor of the environment below cloud tops can either increase or decrease, depending on the cloud top height. The critical factor, which divides these different water vapor variations below cloud tops, is the relative humidity. Clouds hydrate the environment below 16 km, where the air after mixing between cloud and the environmental air does not reach saturation, but clouds dehydrate above 16 km, due to the supersaturation because of the larger temperature drop and the high initial relative humidity. Water vapor above cloud tops has negative anomalies compared to clear skies and suggests another dehydration mechanism. [ABSTRACT FROM AUTHOR]

Published
2010
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49. Localized drugs delivery hydroxyapatite microspheres for osteoporosis therapy

Author

Lee, J. H., Ko, I. H., Jeon, S-H., Chae, J. H., Lee, E. J., and Chang, J. H.

Abstract

This study describes the preparation of hydroxyapatite microspheres for local drugs delivery. The formation of the hydroxyapatite microspheres was initiated by enzymatic decomposition of urea and accomplished by emulsification process (water-in-oil). The microspheres obtained were sintered at 500°C. Scanning electron microscope (SEM) indicated that the microspheres have various porous with random size, which maximizes the surface area. Cytotoxicity was not observed after sintering. Osteoporosis drugs, alendronate and BMP-2, were loaded into HAp microspheres and the releases of both molecules showed sustained releasing profiles.

Published
2011
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50. Coke Formation on KVO<INF>3</INF>−B<INF>2</INF>O<INF>3</INF>/SA5203 Catalysts in the Catalytic Pyrolysis of Naphtha

Author

Lee, W.-H., Jeong, S. M., Chae, J. H., Kang, J.-H., and Lee, W.-J.

Abstract

Coke formation on KVO3−B2O3/SA5203 catalysts during naphtha pyrolysis in a fixed-bed reactor was studied. As the KVO3 loading increased, the amount of coke deposited on KVO3−B2O3/SA5203 decreased, but CO2 formation increased, indicating that KVO3 plays a key role in retarding coke accumulation but not in enhancing the product yields. The coke layers formed on the support and KVO3−B2O3/SA5203 were different in their properties. The coke layer formed on KVO3−B2O3/ SA5203 was free of soluble coke, which dissolves in the CH2Cl2 solution, and consisted of mainly insoluble coke; in contrast, the coke layer formed on the support consisted of both soluble and insoluble coke. The coke layer on KVO3−B2O3/SA5203 catalyst was porous, whereas that on SA5203 was nonporous. KVO3 on the catalyst apparently plays a key role in catalyzing the gasification of soluble coke to CO2.

Published
2004

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