1,510 results on '"Kelley, Richard"'
Search Results
202. Noncoding RNA genes in dosage compensation and imprinting
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Kelley, Richard L. and Kuroda, Mitzi I.
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Cytochemistry -- Research ,RNA -- Genetic aspects ,Dosage compensation (Genetics) -- Research ,Chromatin -- Physiological aspects ,Mice -- Genetic aspects ,Drosophila -- Genetic aspects ,Biological sciences - Abstract
New findings on dosage compensation and how it is controlled in mice, unexpected parallels in Drosophila and four unusual genes involved in dosage compensation are discussed in this review article. The genes may act through RNA products. The list of RNAs required for protein synthesis has grown to many more than three. Now the RNAs associated with chromatin are to be added. Chromosomal RNAs act in cis near sites of their synthesis, implying a novel RNA localization mechanism.
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- 2000
203. The High Resolution Microcalorimeter Soft X-Ray Spectrometer for the Astro-H Mission
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Kelley, Richard L, Mitsuda, Kazuhisa, den Herder, Jan-Willem A, Aarts, Henri J. M, Azzarello, Philipp, Boyce, Kevin R, Brown, Gregory V, Chiao, Meng P, de Vries, Cor P, DiPirro, Michael J, Eckart, Megan E, Ezoe, Yuichiro, Fujimoto, Ryuichi, Gendreau, Keith C, Haas, Daniel, Hoshino, Akio, Ishisaki, Yoshitaka, Kilbourne, Caroline A, Kitamoto, Shunji, Leutenegger, Maurice A, McCammon, Daniel, Murakami, Hiroshi, Murakami, Masahide, Ogawa, Mina, and Okajima, Takashi
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Instrumentation And Photography - Abstract
We are developing the Soft X-Ray Spectrometer for the JAXA Astro-H mission. The instrument features a 5 eV, 36-pixel array of micro calorimeters designed for high spectral resolution from 0.3-12 keV at the focus of an x-ray mirror, providing a field of view of3 x 3 arcmin. The principal components of the spectrometer are the microcalorimeter detector system, a 3-stage ADR and dewar. The dewar is a long-life, hybrid design with a superfluid He cryostat, Joule-Thomson cooler, and Stirling coolers. We describe the present design of the SXS instrument and initial engineering model test results.
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- 2012
204. Search for X-Ray Emission Associated with the Shapley Supercluster with Suzaku
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Mitsuishi, Ikuyuki, Gupta, Anjali, Yamasaki, Noriko Y, Takei, Yoh, Ohashi, Takaya, Sato, Kosuke, Galeazzi, Massimiliano, Henry, J. Patrick, and Kelley, Richard L
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Astronomy - Abstract
Suzaku performed observations of 3 regions in and around the Shapley supercluster: a region located between A3558 and A3556, at approx 0.9 times the virial radii of both clusters, and two other regions at 1 deg and 4 away from the first pointing. The 4 deg -otfset observation was used to evaluate the Galactic foreground emission. We did not detect significant redshifted Oxygen emission lines (O VII and O VIII) in the spectra of all three pointings, after subtracting the contribution of foreground and background emission. An upper limit for the redshifted O VIII Ka line intensity of the warm-hot intergalactic medium (WHIM) is 1.5 x 10(exp -7) photons / s / sq cm / sq arcmin, which corresponds to an overdensity of approx 380 (Z/0.1 Solar Z)(exp -1/2)(L/3 Mpc)(exp -1/2), assuming T = 3 x 10(exp 6) K. We found excess continuum emission in the 1 deg-offset and on-filament regions, represented by thermal models with kT approximates 1 keV and approximates 2 keV, respectively. The redshifts of both 0 and that of the supercluster (0.048) are consistent with the observed spectra. The approx 1 keV emission can be also fitted with Ne-rich Galactic (zero redshift) thin thermal emission. Radial intensity profile of 2 keV component suggests contribution from A3558 and A3556, but with significant steepening of the intensity slope in the outer region of A3558. Finally, we summarized the previous Suzaku search for the WHIM and discussed the feasibility of constraining the WHIM. An overdensity of <400 can be detectable using O VII and O VIII emission lines in a range of 1.4 x 10(exp 6) K < T < 5 x 10(exp 6) K or a continuum emission in a relatively high temperature range T > 5 x 10 (exp 6) K with the Suzaku XIS. The non detection with Suzaku suggests that typical line-of-sight average overdensity is < 400.
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- 2012
205. Cyclopia (synophthalmia) in Smith–Lemli–Opitz syndrome: First reported case and consideration of mechanism
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Weaver, David D., Solomon, Benjamin D., Akin-Samson, Kelly, Kelley, Richard I., and Muenke, Maximilian
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- 2010
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206. The ATHENA X-ray Integral Field Unit (X-IFU)
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Barret, Didier, Thien Lam Trong, den Herder, Jan-Willem, Piro, Luigi, Cappi, Massimo, Huovelin, Juhani, Kelley, Richard, Mas-Hesse, J. Miguel, Mitsuda, Kazuhisa, Paltani, Stephane, Rauw, Gregor, Rozanska, Agata, Wilms, Joern, Bandler, Simon, Barbera, Marco, Barcons, Xavier, Bozzo, Enrico, Ceballos, Maria Teresa, Charles, Ivan, Costantini, Elisa, Decourchelle, Anne, den Hartog, Roland, Duband, Lionel, Duval, Jean-Marc, Fiore, Fabrizio, Gatti, Flavio, Goldwurm, Andrea, Jackson, Brian, Jonker, Peter, Kilbourne, Caroline, Macculi, Claudio, Mendez, Mariano, Molendi, Silvano, Orleanski, Piotr, Pajot, Francois, Pointecouteau, Etienne, Porter, Frederick, Pratt, Gabriel W., Prele, Damien, Ravera, Laurent, Sato, Kosuke, Schaye, Joop, Shinozaki, Keisuke, Thibert, Tanguy, Valenziano, Luca, Valette, Veronique, Vink, Jacco, Webb, Natalie, Wise, Michael, Yamasaki, Noriko, Delcelier-Douchin, Francoise, Mesnager, Jean-Michel, Pontet, Bernard, Pradines, Alice, Branduardi-Raymont, Graziella, Bulbul, Esra, Dadina, Mauro, Ettori, Stefano, Finoguenov, Alexis, Fukazawa, Yasushi, Janiuk, Agnieszka, Kaastra, Jelle, Mazzotta, Pasquale, Miller, Jon, Miniutti, Giovanni, Naze, Yael, Nicastro, Fabrizio, Sciortino, Salvatore, Simionescu, Aurora, Torrejon, Jose Miguel, Geoffray, Herve, Peille, Philippe, Aicardi, Corinne, Andre, Jerome, Garrido, Gonzalo Campos, Clenet, Antoine, Daniel, Christophe, Etcheverry, Christophe, Frezouls, Benot, Gloaguen, Emilie, Hervet, Gilles, Jolly, Antoine, Ledot, Aurelien, Maussang, Irwin, Paillet, Alexis, Schmisser, Roseline, Travert, Jean-Michel, Vella, Bruno, Damery, Jean-Charles, Boyce, Kevin, DiPirro, Michael, Lotti, Simone, Schwander, Denis, Smith, Stephen, van Leeuwen, Bert-Joost, van Weers, Henk, Clerc, Nicolas, Cobo, Beatriz, Dauser, Thomas, de Plaa, Jelle, Kirsch, Christian, Cucchetti, Edoardo, Eckart, Megan, Ferrando, Philippe, Natalucci, Lorenzo, European Commission, Ministerio de Economía y Competitividad (España), Fonds de la Recherche Scientifique (Fédération Wallonie-Bruxelles), High Energy Astrophys. & Astropart. Phys (API, FNWI), DenHerder, JWA, Nikzad, S, Nakazawa, K, Department of Physics, Barret, Didier, Lam Trong, Thien, den Herder, Jan-Willem, Piro, Luigi, Cappi, Massimo, Houvelin, Juhani, Kelley, Richard, Mas-Hesse, J. Miguel, Mitsuda, Kazuhisa, Paltani, Stéphane, Rauw, Gregor, Rozanska, Agata, Wilms, Joern, Bandler, Simon, Barbera, Marco, Barcons, Xavier, Bozzo, Enrico, Ceballos, Maria Teresa, Charles, Ivan, Costantini, Elisa, Decourchelle, Anne, den Hartog, Roland, Duband, Lionel, Duval, Jean-Marc, Fiore, Fabrizio, Gatti, Flavio, Goldwurm, Andrea, Jackson, Brian, Jonker, Peter, Kilbourne, Caroline, Macculi, Claudio, Mendez, Mariano, Molendi, Silvano, Orleanski, Piotr, Pajot, Françoi, Pointecouteau, Etienne, Porter, Frederick, Pratt, Gabriel W., Prêle, Damien, Ravera, Laurent, Sato, Kosuke, Schaye, Joop, Shinozaki, Keisuke, Thibert, Tanguy, Valenziano, Luca, Valette, Véronique, Vink, Jacco, Webb, Natalie, Wise, Michael, Yamasaki, Noriko, Douchin, Françoise, Mesnager, Jean-Michel, Pontet, Bernard, Pradines, Alice, Branduardi-Raymont, Graziella, Bulbul, Esra, Dadina, Mauro, Ettori, Stefano, Finoguenov, Alexi, Fukazawa, Yasushi, Janiuk, Agnieszka, Kaastra, Jelle, Mazzotta, Pasquale, Miller, Jon, Miniutti, Giovanni, Naze, Yael, Nicastro, Fabrizio, Scioritino, Salavtore, Simonescu, Aurora, Torrejon, Jose Miguel, Frezouls, Benoit, Geoffray, Hervé, Peille, Philippe, Aicardi, Corinne, André, Jérôme, Daniel, Christophe, Clénet, Antoine, Etcheverry, Christophe, Gloaguen, Emilie, Hervet, Gille, Jolly, Antoine, Ledot, Aurélien, Paillet, Irwin, Schmisser, Roseline, Vella, Bruno, Damery, Jean-Charle, Boyce, Kevin, Dipirro, Mike, Lotti, Simone, Schwander, Deni, Smith, Stephen, Van Leeuwen, Bert-Joost, van Weers, Henk, Clerc, Nicola, Cobo, Beatriz, Dauser, Thoma, Kirsch, Christian, Cucchetti, Edoardo, Eckart, Megan, Ferrando, Philippe, Natalucci, Lorenzo, and Fonds de la Recherche Scientifique (Belgique)
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Point spread function ,Photon ,Astrophysics::High Energy Astrophysical Phenomena ,Field of view ,Athena ,Instrumentation ,Space telescopes ,X-ray Integral Field Unit ,X-ray spectroscopy ,Electronic, Optical and Magnetic Materials ,Condensed Matter Physics ,Computer Science Applications1707 Computer Vision and Pattern Recognition ,Applied Mathematics ,Electrical and Electronic Engineering ,Condensed Matter Physic ,Large format ,01 natural sciences ,7. Clean energy ,law.invention ,Telescope ,Physics::Popular Physics ,Settore FIS/05 - Astronomia E Astrofisica ,Optics ,law ,Physics::Plasma Physics ,0103 physical sciences ,Electronic ,Optical and Magnetic Materials ,Spectral resolution ,010306 general physics ,010303 astronomy & astrophysics ,Physics ,Spectrometer ,business.industry ,Electronic, Optical and Magnetic Material ,Detector ,Astrophysics::Instrumentation and Methods for Astrophysics ,115 Astronomy, Space science ,Physics::History of Physics ,Applied Mathematic ,Space telescope ,business - Abstract
Event: SPIE Astronomical Telescopes + Instrumentation, 2018, Austin, Texas, United States., The X-IFU will be provided by an international consortium led by France, The Netherlands and Italy, with further ESA member state contributions from Belgium, Finland, Germany, Poland, Spain, Switzerland and two international partners from the United States and Japan: et al., The X-ray Integral Field Unit (X-IFU) is the high resolution X-ray spectrometer of the ESA Athena X-ray observatory. Over a field of view of 5’ equivalent diameter, it will deliver X-ray spectra from 0.2 to 12 keV with a spectral resolution of 2.5 eV up to 7 keV on ∼ 5” pixels. The X-IFU is based on a large format array of super-conducting molybdenum-gold Transition Edge Sensors cooled at ∼ 90 mK, each coupled with an absorber made of gold and bismuth with a pitch of 249 μm. A cryogenic anti-coincidence detector located underneath the prime TES array enables the non X-ray background to be reduced. A bath temperature of ∼ 50 mK is obtained by a series of mechanical coolers combining 15K Pulse Tubes, 4K and 2K Joule-Thomson coolers which pre-cool a sub Kelvin cooler made of a 3He sorption cooler coupled with an Adiabatic Demagnetization Refrigerator. Frequency domain multiplexing enables to read out 40 pixels in one single channel. A photon interacting with an absorber leads to a current pulse, amplified by the readout electronics and whose shape is reconstructed on board to recover its energy with high accuracy. The defocusing capability offered by the Athena movable mirror assembly enables the X-IFU to observe the brightest X-ray sources of the sky (up to Crab-like intensities) by spreading the telescope point spread function over hundreds of pixels. Thus the X-IFU delivers low pile-up, high throughput (< 50%), and typically 10 eV spectral resolution at 1 Crab intensities, i.e. a factor of 10 or more better than Silicon based X-ray detectors. In this paper, the current X-IFU baseline is presented, together with an assessment of its anticipated performance in terms of spectral resolution, background, and count rate capability. The X-IFU baseline configuration will be subject to a preliminary requirement review that is scheduled at the end of 2018., We acknowledge support from the Athena Science Study Team, the Athena Working Group Chairs, the Athena Topical Panel Chairs and the Topical Panel members in strengthening the X-IFU top level performance requirements. Particular thanks go to: E. Rasia, V. Biffi, S. Borgani and K. Dolag for providing cosmological hydrodynamic simulations of a cluster used to produce simulation of X-IFU observation presented in Fig. 2; P.T. O'Brien for assistance with Sect. 2.2.1, A.C. Fabian and C. Pinto for providing inputs for Figure 1. We also thank the ESA project team, and in particular Mark Ayre and Ivo Ferreira, for their work on the assessment of the ToO efficiency requirement. The Italian contribution to X-IFU is supported through the ASI contractn. 2015-046-R.0. XB, MTC and BC acknowledge nancial support by MINECO through grant ESP2014-53672-C3-1-P. A.R., P.O, and A.J. were supported by Polish NSC grants: 2015/17/B/ST9/03422 and 2015/18/M/ST9/00541. GR, ER, YN, and PJ acknowledges support by FNRS and Prodex (Belspo). This work was supported by the French Space Agency (CNES).
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- 2018
207. Specific isoforms of squid, a 'Drosophila' hnRNP, perform distinct roles in gurken localization during oogenesis
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Norvell, Amanda, Kelley, Richard L., Wehr, Kristina, and Schupbach, Trudi
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Oogenesis -- Research ,RNA -- Research ,Genetic regulation -- Research ,Biological sciences - Abstract
Squid (sqd), a Drosophila melanogaster gene, is key in localization and translational regulation of mRNAs. Mutations to sqd proteins can cause sterility in females due to gurken mislocation during oogenesis. SqdA is primarily cytoplasmic, while SqdB and SqdS are primarily located in germ-line nuclei. Differential nuclear accumulation corresponds with association of nuclear import proteins.
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- 1999
208. In-orbit Operation of the Soft X-ray Spectrometer onboard the Hitomi satellite
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Kelley, Richard L., den, Herder Jan-Willem, Bialas, Thomas G., Boyce, Kevin R., Chiao, Meng P., de, Vries Cor P., DiPirro, Michael J., Eckart, Megan E., Kilbourne, Caroline A., Leutenegger, Maurice A., Masters, Candace M., Porter, Frederick S., Savinell, Joseph C., Shirron, Peter J., Sneiderman, Gary A., Tsujimoto, Masahiro, Mitsuda, Kazuhisa, Ezoe, Yuichiro, Fujimoto, Ryuichi, Hoshino, Akio, Ishikawa, Kumi, Ishisaki, Yoshitaka, Koyama, Shu, Mitsuishi, Ikuyuki, Noda, Hirofumi, Okajima, Takashi, Okamoto, Atsushi, Porter, Frederic S., Sato, Kosuke, Sato, Yohichi, Sawada, Makoto, Seta, Hiromi, Takei, Yoh, Tamagawa, Toru, Tashiro, Makoto S., Watanabe, Tomomi, Yamada, Shinya, Yamasaki, Noriko Y., and Yatsu, Yoichi
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Physics ,Soft x ray ,x-ray microcalorimeter ,Spectrometer ,Mechanical Engineering ,Space operations ,Astronomy and Astrophysics ,02 engineering and technology ,021001 nanoscience & nanotechnology ,01 natural sciences ,Electronic, Optical and Magnetic Materials ,010309 optics ,soft x-ray spectrometer ,Short lifetime ,Hitomi ,Space and Planetary Science ,Control and Systems Engineering ,0103 physical sciences ,Orbit (dynamics) ,Satellite ,in-orbit operations ,0210 nano-technology ,Instrumentation ,Remote sensing - Abstract
形態: カラー図版あり, Physical characteristics: Original contains color illustrations, Accepted: 2017-10-13, 資料番号: PA1810023000
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- 2017
209. Characterization of lymphoblast mitochondria from patients with Barth syndrome
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Xu, Yang, Sutachan, John J, Plesken, Heide, Kelley, Richard I, and Schlame, Michael
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- 2005
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210. High Spectral Resolution, High Cadence, Imaging X-Ray Microcalorimeters for Solar Physics
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Bandler, Simon R, Bailey, Catherine N, Bookbinder, Jay A, DeLuca, Edward E, Chervenak, Jay A, Eckart, Megan E, Finkbeiner, Fred M, Kelley, Daniel P, Kelley, Richard L, Kilbourne, Caroline A, Porter, Frederick S, Sadleir, Jack E, Smith, Stephen J, and Smith, Randall K
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Solar Physics - Abstract
High spectral resolution, high cadence, imaging x-ray spectroscopy has the potential to revolutionize the study of the solar corona. To that end we have been developing transition-edge-sensor (TES) based x-ray micro calorimeter arrays for future solar physics missions where imaging and high energy resolution spectroscopy will enable previously impossible studies of the dynamics and energetics of the solar corona. The characteristics of these x-ray microcalorimeters are significantly different from conventional micro calorimeters developed for astrophysics because they need to accommodate much higher count rates (300-1000 cps) while maintaining high energy resolution of less than 4 eV FWHM in the X-ray energy band of 0.2-10 keV. The other main difference is a smaller pixel size (less than 75 x 75 square microns) than is typical for x-ray micro calorimeters in order to provide angular resolution less than 1 arcsecond. We have achieved at energy resolution of 2.15 eV at 6 keV in a pixel with a 12 x 12 square micron TES sensor and 34 x 34 x 9.1 micron gold absorber, and a resolution of 2.30 eV at 6 keV in a pixel with a 35 x 35 micron TES and a 57 x 57 x 9.1 micron gold absorber. This performance has been achieved in pixels that are fabricated directly onto solid substrates, ie. they are not supported by silicon nitride membranes. We present the results from these detectors, the expected performance at high count-rates, and prospects for the use of this technology for future Solar missions.
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- 2010
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211. Status of resolve instrument onboard X-Ray Imaging and Spectroscopy Mission (XRISM)
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den Herder, Jan-Willem A., Nikzad, Shouleh, Nakazawa, Kazuhiro, Ishisaki, Yoshitaka, Kelley, Richard L., Awaki, Hisamitsu, Balleza, Jesus C., Barnstable, Kim R., Bialas, Thomas G., Boissay-Malaquin, Rozenn, Brown, Gregory V., Canavan, Edgar R., Cumbee, Renata S., Carnahan, Timothy M., Chiao, Meng P., Comber, Brian J., Costantini, Elisa, den Herder, Jan-Willem, Dercksen, Johannes, de Vries, Cor P., DiPirro, Michael J., Eckart, Megan E., Ezoe, Yuichiro, Ferrigno, Carlo, Fujimoto, Ryuichi, Gorter, Nathalie, Graham, Steven M., Grim, Martin, Hartz, Leslie S., Hayakawa, Ryota, Hayashi, Takayuki, Hell, Natalie, Hoshino, Akio, Ichinohe, Yuto, Ishida, Manabu, Ishikawa, Kumi, James, Bryan L., Kenyon, Steven J., Kilbourne, Caroline A., Kimball, Mark O., Kitamoto, Shunji, Leutenegger, Maurice A., Maeda, Yoshitomo, McCammon, Dan, Miko, Joseph J., Mizumoto, Misaki, Okajima, Takashi, Okamoto, Atsushi, Paltani, Stephane, Porter, Frederick S., Sato, Kosuke, Sato, Toshiki, Sawada, Makoto, Shinozaki, Keisuke, Shipman, Russell, Shirron, Peter J., Sneiderman, Gary A., Soong, Yang, Szymkiewicz, Richard, Szymkowiak, Andrew E., Takei, Yoh, Tamura, Keisuke, Tsujimoto, Masahiro, Uchida, Yuusuke, Wasserzug, Stephen, Witthoeft, Michael C., Wolfs, Rob, Yamada, Shinya, and Yasuda, Susumu
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- 2022
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212. Results of accelerometer monitor in the ground testing of Resolve x-ray microcalorimeter instrument onboard XRISM
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Holland, Andrew D., Beletic, James, Imamura, Ryuta, Tsujimoto, Masahiro, Awaki, Hisamitsu, Chiao, Meng P., Fujimoto, Ryuichi, Ishisaki, Yoshitaka, Kelley, Richard L., Kilbourne, Caroline A., Porter, Frederick S., Sawada, Makoto, Sneiderman, Gary A., Takei, Yoh, and Yamada, Shinya
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- 2022
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213. X線分光撮像衛星 (XRISM) 搭載 Resolve の開発状況
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KELLEY, Richard L., BIALAS, Thomas G., BROWN, Gregory V., CHIAO, Meng P., CONSTANTINI, Elisa, DENHERDER, Jan-Willem, DIPIRRO, Michael J., ECKART, Megan E., FERRIGNO, Carlo, GRAHAM, Steven M., GRIM, Martin, KILBOURNE, Caroline A., LEUTENEGGER, Maurice A., MCCMMON, Dan, PALTANI, Stephane, PORTER, F. Scott, SHIRRON, Peter J., SNEIDERMAN, Gary A., SOONG, Yang, SZYMKOWIAK, Andrew E., VRIES, Cor P. de, ISHISAKI, Yoshitaka, AKAMATSU, Hiroki, AWAKI, HIisamitsu, COSTANTINI, Elisa, DEN, HERDER Jan-William, EZOE, Yuichiro, FUJIMOTO, Ryuichi, FURUZAWA, Akihiro, HAYASHI, Takayuki, HORIUCHI, Takafumi, HOSHINO, Akio, ICHINOHE, Yuto, IIZUKA, Ryo, ISHIBASHI, Kazunori, ISHIDA, Manabu, ISHIKAWA, Kumi, KITAMOTO, Shunji, MAEDA, Yoshitomo, MCCAMMON, Dan, MITSUISHI, Ikuyuki, MORI, Hideyuki, OHASHI, Takaya, OKAJIMA, Takashi, SATO, Kosuke, SAWADA, Makoto, SETA, Hiromi, SHIBANO, Yasuko, TAKEI, Yoh, TAMAGAWA, Toru, TSUJIMOTO, Masahiro, UCHIDA, Yuusuke, YAMADA, Shinya, YAMASAKI, Noriko Y., YASUDA, Susumu, and YOSHIOKA, Nasa
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第19回宇宙科学シンポジウム (2019年1月9日-10日. 宇宙航空研究開発機構宇宙科学研究所(JAXA)(ISAS)相模原キャンパス), 相模原市, 神奈川県, 19th Space Science Symposium (January 9-10, 2019. Institute of Space and Astronautical Science, Japan Aerospace Exploration Agency(JAXA)(ISAS)Sagamihara Campus), Sagamihara, Kanagawa Japan, 著者人数: 53名, 資料番号: SA6000144056, レポート番号: P-020
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- 2019
214. The Synthetic Search for Better Thermoelectrics
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Reynolds, Thomas K., primary, Bales, Joshua G., additional, Kelley, Richard F., additional, and DiSalvo, Francis J., additional
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- 2003
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215. Thermal Transport Model for Heat Sink Design
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Chervenak, James A, Kelley, Richard L, Brown, Ari D, Smith, Stephen J, and Kilbourne, Caroline a
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Man/System Technology And Life Support - Abstract
A document discusses the development of a finite element model for describing thermal transport through microcalorimeter arrays in order to assist in heat-sinking design. A fabricated multi-absorber transition edge sensor (PoST) was designed in order to reduce device wiring density by a factor of four. The finite element model consists of breaking the microcalorimeter array into separate elements, including the transition edge sensor (TES) and the silicon substrate on which the sensor is deposited. Each element is then broken up into subelements, whose surface area subtends 10 10 microns. The heat capacity per unit temperature, thermal conductance, and thermal diffusivity of each subelement are the model inputs, as are the temperatures of each subelement. Numerical integration using the Finite in Time Centered in Space algorithm of the thermal diffusion equation is then performed in order to obtain a temporal evolution of the subelement temperature. Thermal transport across interfaces is modeled using a thermal boundary resistance obtained using the acoustic mismatch model. The document concludes with a discussion of the PoST fabrication. PoSTs are novel because they enable incident x-ray position sensitivity with good energy resolution and low wiring density.
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- 2009
216. Dehydrosteroid measurements in maternal urine or serum for the prenatal diagnosis of Smith–Lemli–Opitz syndrome (SLOS)
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Shackleton, Cedric H.L., Marcos, Josep, Palomaki, Glenn E., Craig, Wendy Y., Kelley, Richard I., Kratz, Lisa E., and Haddow, James E.
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- 2007
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217. RSH/Smith-Lemli-Opitz syndrome: mutations and metabolic morphogenesis
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Kelley, Richard I.
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Genetic disorders -- Research ,Metabolic diseases -- Genetic aspects ,Mutation (Biology) -- Research ,Birth defects -- Research ,Cellular signal transduction -- Research ,Sterols -- Physiological aspects ,Biochemical genetics -- Research ,Biological sciences - Abstract
Smith-Lemli-Opitz syndrome (SLOS), also called RSH syndrome, is reviewed in relation to metabolic morphogenesis and the mutations involved. It is an autosomal recessive, multiple anomaly syndrome that is characterized by genital malformation in males, polydactyly, and craniofacial anomalies including cleft palate. Patients with this syndrome have an increased blood levels of 7-dehydrocholesterol (7DHC), increased by as much as a factor of 2,000. The molecule is the immediate precursor of cholesterol in the Kandutch-Russell cholesterol biosynthesis pathway. The clinical phenotype has been expanded and some near-normal children are included as well as some very defective fetuses that die before birth. Patients' lives are dramatically improved when the cholesterol-deficiency syndrome is treated. Other biochemically undiagnosed malformation syndromes will be investigated for disorders of cholesterol biosynthesis but mutations that bring many malformations have been those of of homeobox genes and other transcription regulators and signaling systems.
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- 1998
218. Identifying Smith-Lemli-Opitz syndrome in conjunction with prenatal screening for Down syndrome
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Craig, Wendy Y., Haddow, James E., Palomaki, Glenn E., Kelley, Richard I., Kratz, Lisa E., Shackleton, Cedric H. L., Marcos, Josep, Tint, Stephen G., MacRae, Andrew R, Nowaczyk, Malgorzata J., Kloza, Edward M., Irons, Mira B., and Roberson, Marie
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- 2006
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219. Drosophila male-specific lethal-2 protein: structure/function analysis and dependence on MSL-1 for chromosome association
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Lyman, Laura M., Copps, Kyle, Rastelli, Luca, Kelley, Richard L., and Kuroda, Mitzi I.
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Drosophila -- Genetic aspects ,X chromosome -- Research ,Chromosomal proteins -- Research ,Biological sciences - Abstract
MSL-2 is required for the male-specific assembly of a dosage compensation regulatory complex on the X chromosome of Drosophila melanogaster. We found that MSL-2 binds in a reproducible, partial pattern to the male X chromosome in the absence of MLE or MSL-3, or when ectopically expressed at a low level in females. Moreover, the pattern of MSL-2 binding corresponds precisely in each case to that of MSL-1, suggesting that the two proteins function together to associate with the X. Consistent with this hypothesis, we isolated EMS-induced loss of function msl-1 and msl-2 alleles in a screen for suppressors of the toxic effects of MSL-2 expression in females. We also used site-directed mutagenesis to determine the importance of the MSL-2 RING finger domain and second cysteine-rich motif. The mutations, including those in conserved zinc coordinating cysteines, confirm that the RING finger is essential for MSL-2 function, while suggesting a less stringent requirement for an intact second motif.
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- 1997
220. Sex lethal controls dosage compensation in Drosophila by a non-spicing mechanism
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Kelley, Richard L., Wang, Jiwu, Bell, Leslie, and Kuroda, Mitzi I.
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Sex-linkage (Genetics) -- Research ,RNA splicing -- Research ,Sex determination, Genetic -- Research ,Environmental issues ,Science and technology ,Zoology and wildlife conservation - Abstract
Genetic research indicates female-specific ribonucleic acid-binding (RNA) sex lethal protein (SXL), which controls messenger RNA splicing in Drosophila, is also able to repress the translation of RNA transcripts in the process of sex determination. Deoxyribonucleic acid genetic sequences, photographs and diagrams illustrate the research findings.
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- 1997
221. Practical Considerations for Optimizing Position Sensitivity in Arrays of Position-sensitive TES's
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Smith, Stephen J, Bandler, Simon R, Figueroa-Feliciano, Encetali, Iyomoto, Naoko, Kelley, Richard L, Kilbourne, Caroline A, Porder, Frederick S, and Sadleir, John E
- Subjects
Physics (General) - Abstract
We are developing Position-Sensitive Transitions-Edge Sensors (PoST's) for future X-ray astronomy missions such as NASA's Constellation-X. The PoST consists of one or more Transitions Edge Sensors (TES's) thermally connected to a large X-ray absorber, which through heat diffusion, gives rise to position dependence. The development of PoST's is motivated by the desire to achieve the largest the focal-plan coverage with the fewest number of readout channels. In order to develop a practical array, consisting of an inner pixellated core with an outer array of large absorber PoST's, we must be able to simultaneously read out all (-1800) channels in the array. This is achievable using time division multiplexing (TDM), but does set stringent slew rate requirements on the array. Typically, we must damp the pulses to reduce the slew rate of the input signal to the TDM. This is achieved by applying a low-pass analog filter with large inductance to the signal. This attenuates the high frequency components of the signal, essential for position discrimination in PoST's, relative to the white noise of the readout chain and degrades the position sensitivity. Using numerically simulated data, we investigate the position sensing ability of typical PoST designs under such high inductance conditions. We investigate signal-processing techniques for optimal determination of the event position and discuss the practical considerations for real-time implementation.
- Published
- 2007
222. Heat Sinking, Cross Talk, and Temperature Stability for Large, Close-Packed Arrays of Microcalorimeters
- Author
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Imoto, Naoko, Bandler, SImon, Brekosky, Regis, Chervenak, James, Figueroa-Felicano, Enectali, Finkbeiner, Frederick, Kelley, Richard, Kilbourne, Caroline, Porter, Frederick, Sadleir, Jack, and Smith, Stephen
- Subjects
Electronics And Electrical Engineering - Abstract
We are developing large, close-packed arrays of x-ray transition-edge sensor (TES) microcalorimeters. In such a device, sufficient heat sinking is important to to minimize thermal cross talk between pixels and to stabilize the bath temperature for all pixels. We have measured cross talk on out 8 x 8 arrays and studied the shape and amount of thermal crosstalk as a function of pixel location and efficiency of electrothermal feedback. In this presentation, we will compare measurements made on arrays with and without a backside, heat-sinking copper layer, as well as results of devices on silicon-nitride membranes and on solid substrates, and we will discuss the implications for energy resolution and maximum count rate. We will also discuss the dependence of pulse height upon bath temperature, and the measured and required stability of the bath temperature.
- Published
- 2007
223. Characterizing the Superconducting-to-Normal Transition in Mo/Au Transition-Edge Sensor Bilayers
- Author
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Smith, Stephen J, Bandler, Simon R, Brown, Ari. -D, Chervenak, Jay A, Finkbeiner, Fred M, Iyomoto, Naoko, Kelley, Richard L, Kilbourne, Caroline A, Porter, Frederick S, and Sadleir, John E
- Subjects
Astronomy - Abstract
We are developing arrays of Mo/Au bilayer transition-edge sensors (TES's) for applications in future X-ray astronomy missions such as NASA's Constellation-X. The physical properties of the superconducting-to-normal transition in our TES bilayers, while often reproducible and characterized, are not well understood. The addition of normal metal features on top of the bilayer are found to change the shape and temperature of the transition, and they typically reduce the unexplained 'excess' noise. In order to understand and potentially optimize the properties of the transition, we have been studying the temperature, widths and current dependence of these transitions. We report on the characterization of devices both deposited on silicon substrates and suspended on thin silicon nitride membranes. This includes key device parameters such as the logarithmic resistance sensitivity with temperature alpha, and the logarithmic resistance sensitivity with current beta, of the phase-transition. We investigate alpha and beta as a function of current, both at fixed and varying bias points in the transition. Using Ginzburg-Landau theory for the current dependence of the superconducting transition temperature, we investigate the relationship between alpha and beta and compare our measured and theoretical estimates.
- Published
- 2007
224. Modelling of TES X-ray Microcalorimeters with a Novel Absorber Design
- Author
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Iyomoto, Naoko, Bandler, Simon, Brefosky, Regis, Brown, Ari, Chervenak, James, Figueroa-Feliciano, Enectali, Finkbeiner, Frederick, Kelley, Richard, Kilbourne, Caroline, Lindeman, Mark, Porter, Frederick, Saab, Tarek, Sadleir, Jack, and Smith, Stephen
- Subjects
Electronics And Electrical Engineering - Abstract
Our development of a novel x-ray absorber design that has enabled the incorporation of high-conductivity electroplated gold into our absorbers has yielded devices that not only have achieved breakthrough performance at 6 keV, but also are extraordinarily well modelled. We have determined device parameters that reproduce complex impedance curves and noise spectra throughout transition. Observed pulse heights, decay time and baseline energy resolution were in good agreement with simulated results using the same parameters. In the presentation, we will show these results in detail and we will also show highlights of the characterization of our gold/bismuth-absorber devices. We will discuss possible improvement of our current devices and expected performance of future devices using the modelling results.
- Published
- 2007
225. A prototype kinetic inductance thermometer for X-ray calorimetry
- Author
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Audley, Michael D., Kelley, Richard L., and Rawley, Gayle L.
- Published
- 1993
- Full Text
- View/download PDF
226. Absorber Materials for Transition-Edge Sensor X-ray Microcalorimeters
- Author
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Brown, Ari-David, Bandler, Simon, Brekosky, Regis, Chervenak, James, Figueroa-Feliciano, Enectali, Finkbeiner, Fred, Sadleir, Jack, Iyomoto, Naoko, Kelley, Richard, Kilbourne, Caroline, Porter, F. Scott, Smith, Stephen, Saab, Tarek, and Sadleir, Jac
- Subjects
Astronomy - Abstract
Arrays of superconducting transition-edge sensors (TES) can provide high spatial and energy resolution necessary for x-ray astronomy. High quantum efficiency and uniformity of response can be achieved with a suitable absorber material, in which absorber x-ray stopping power, heat capacity, and thermal conductivity are relevant parameters. Here we compare these parameters for bismuth and gold. We have fabricated electroplated gold, electroplated gold/electroplated bismuth, and evaporated gold/evaporated bismuth 8x8 absorber arrays and find that a correlation exists between the residual resistance ratio (RRR) and thin film microstructure. This finding indicates that we can tailor absorber material conductivity via microstructure alteration, so as to permit absorber thermalization on timescales suitable for high energy resolution x-ray microcalorimetry. We show that by incorporating absorbers possessing large grain size, including electroplated gold and electroplated gold/electroplated bismuth, into our current Mo/Au TES, devices with tunable heat capacity and energy resolution of 2.3 eV (gold) and 2.1 eV (gold/bismuth) FWHM at 6 keV have been fabricated.
- Published
- 2007
227. Sex and Repression
- Author
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Kuroda, Mitzi I. and Kelley, Richard L.
- Published
- 1999
228. Performance of compact TES arrays with integrated high-fill-fraction X-ray absorbers
- Author
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Lindeman, Mark A., Bandler, Simon, Brekosky, Regis P., Chervenak, James A., Figueroa-Feliciano, Enectali, Finkbeiner, Fred M., Kelley, Richard L., Saab, Tarek, Stahle, Caroline K., and Talley, D.J.
- Published
- 2004
- Full Text
- View/download PDF
229. Position-sensitive transition edge sensor modeling and results
- Author
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Hammock, Christina, Figueroa-Feliciano, Enectali, Apodaca, Emmanuel, Bandler, Simon, Boyce, Kevin, Chervenak, Jay, Finkbeiner, Fred, Kelley, Richard, Lindeman, Mark, Porter, Scott, Saab, Tarek, and Stahle, Caroline
- Published
- 2004
- Full Text
- View/download PDF
230. The X-ray microcalorimeter instruments on the Astro-E2 and Constellation-X X-ray observatories
- Author
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Kelley, Richard L.
- Published
- 2004
- Full Text
- View/download PDF
231. Electronic transport and magnetic properties of a new nickel antimonide telluride, Ni 2SbTe 2
- Author
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Reynolds, Thomas K., Kelley, Richard F., and DiSalvo, Francis J.
- Published
- 2004
- Full Text
- View/download PDF
232. The X-ray microcalorimeter spectrometer (XMS): a reference cryogenic instrument for Constellation-X
- Author
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Whitehouse, Paul L, Shirron, Peter J, and Kelley, Richard L
- Published
- 2004
- Full Text
- View/download PDF
233. Poisson vs. Gaussian statistics for sparse X-ray data : Application to the soft X-ray spectrometer
- Author
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Yamada, Shinya, Axelsson, Magnus, Ishisaki, Yoshitaka, Konami, Saori, Takemura, Nozomi, Kelley, Richard L., Kilbourne, Caroline A., Leutenegger, Maurice A., Porter, F. Scott, Eckart, Megan E., Szymkowiak, Andrew, Yamada, Shinya, Axelsson, Magnus, Ishisaki, Yoshitaka, Konami, Saori, Takemura, Nozomi, Kelley, Richard L., Kilbourne, Caroline A., Leutenegger, Maurice A., Porter, F. Scott, Eckart, Megan E., and Szymkowiak, Andrew
- Abstract
Reliable results when fitting X-ray data require proper consideration of the statistics involved. We probe the impact of Gaussian versus Poisson statistics at low count levels using both the standard chi(2) method and maximum likelihood based on Poisson studied and quantified through simulated spectra with known properties. We then test the results through analysis of Mn K alpha calibration data taken with the flight spare microcalorimeter for the Hitomi soft X-ray spectrometer. Through comparison with simulations, our results show that the chi(2) method tends to give overly optimistic estimates of the detector energy resolution, in particular when there are few counts. Given an energy resolution of similar to 5eV and a line with about 100 photons, the line width becomes similar to 10% lower in the chi(2) method than in Poisson statistics. This is a consequence of the uncertainties being dominated by counting statistics, and therefore highlights the need to choose the appropriate fit statistic., QC 20190920
- Published
- 2019
- Full Text
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234. The Suzaku High Resolution X-ray Spectrometer
- Author
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Kelley, Richard L, Mitsuda, Kazuhisa, Allen, Christine A, Arsenovic, Peter, Bialas, Thomas G, Boyce, Kevin R, Boyle, Robert F, Breon, Susan R, Brown, Gregory V, Cottam, Jean, Dipirro, Michael J, Fujimoto, Ryuichi, Furusho, Tae, Gendreau, Keith, Gochar, Gene G, Gonzalez, Oscar, and Hirabayashi, Masayuki
- Subjects
Instrumentation And Photography - Abstract
The high resolution X-Ray Spectrometer (XRS) has been designed to provide the Suzaku Observatory with very high spectral resolution, non-dispersive spectroscopy from 0.3 to 12 keV. This energy range encompasses the most diagnostically-rich part of the x-ray band. The sensor consists of a 32 channel array of x-ray of microcalorimeters, each with an energy resolution of about 6 eV. The very low temperature required for operation of the array (60 mK) is provided by a four-stage cooling system containing a single stage ADR, superfluid He Cryostat, solid Ne Dewar, and a single-stage Stirling-cycle cooler. The Suzaku/XRS is the first orbiting x-ray microcalorimeter spectrometer and has been designed to last more than three years in orbit. The early verification phase of the mission demonstrated that the instrument was working properly and that the cryogen consumption rate was low enough to ensure a mission lifetime exceeding 3 years. However, the liquid He cryogen was completely vaporized two weeks after opening the dewar guard vacuum vent. The problem has been traced to inadequate venting of the dewar He and Ne gases out of the spacecraft into space. In this paper we present the design of the XRS instrument and describe the in-flight performance.
- Published
- 2006
235. On-orbit Performance Of The Suzaku/XRS
- Author
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Kelley, Richard
- Subjects
Instrumentation And Photography - Abstract
The Suzaku high-resolution X-Ray Spectrometer (XRS) is the first orbiting x-ray microcalorimeter. This instrument, which maintains the sensor at less than 0.1K and utilizes liquid He and solid Ne cryogens, is capable of providing very high, non-dispersive x-ray spectroscopy. The microcalorimeter approach enables increasing spectral resolving power toward increasing energy. This fundamental characteristic makes it the instrument of choice for a variety of spectral diagnostics that can be uniquely obtained from studies of Fe K-line features common in such objects as cluster of galaxies and active galaxies, to name only two important classes out of many. The array aspect allows for imaging spectroscopy of extended sources without spectral degradation. Unfortunately, a design flaw in the XRS dewar/spacecraft interface caused the premature loss of liquid helium and rendered the instrument miusable. Nonetheless, during the three weeks following the launch of Suzaku, the instrument performed extremely well and demonstrated that the basic microcalorimeter concept is sound for space applications. An energy resolution of 7 eV FWHM at 6keV was obtained in orbit as measured using internal calibration sources. We will describe the XRS design and operation, explain what happened, and then present some lessons learned for future space missions utilizing this powerful technology.
- Published
- 2006
236. Precision Fe K-Alpha and Fe K-Beta Line Spectroscopy of the Seyfert 1.9 Galaxy NGC 2992 with Suzaku
- Author
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Yaqoob, Tahir, Murphy, Kendrah D, Griffiths, Richard E, Haba, Yoshito, Inoue, Hajime, Itoh, Takeshi, Kelley, Richard, Kokubun, Motohide, Markowitz, Alex, Mushotzky, Richard, Okajima, Takashi, Ptak, Andrew, Reeves, James, Selemitos, Peter J, Takahashi, Tadayuki, and Terashima, Yuichi
- Subjects
Astrophysics - Abstract
We present detailed time-averaged X-ray spectroscopy in the 0.5-10 keV band of the Seyfert 1.9 galaxy NGC 2992 with the Suzaku X-ray Imaging Spectrometers (XIS). The source had a factor approximately 3 higher 2-10 keV flux (approximately 1.2 x l0(exp -11) erg per square cm per s) than the historical minimum and a factor approximately 7 less than the historical maximum. The XIS spectrum of NGC 2992 can be described by several components. There is a primary continuum, modeled as a power-law with a photon index of Gamma = 1.57(sup +0.06) (sup -0.03) that is obscured by a Compton-thin absorber with a column density of 8.01(sup +0.6) (sup -0.5)x l0 (exp 21) per square cm. . There is another, weaker, unabsorbed power-law component (modeled with the same slope as the primary), that is likely to be due to the primary continuum being electron-scattered into our line-of-sight by a region extended on a scale of hundreds of parsecs. We measure the Thomson depth of the scattering zone to be Tau = 0.072 +/- 0.021. An optically-thin thermal continuum emission component, which probably originates in the same extended region, is included in the model and yields a temperature and luminosity of KT = 0.656(sup +0.088) (sup -0.0.61) keV and approximately 1.2 +/- 0.4 x l0 (exp 40) erg per s respectively. We detect an Fe K emission complex which we model with broad and narrow lines and we show that the intensities of the two components are decoupled at a confidence level > 3 sigma. The broad Fe K alpha line has an equivalent width of 118(sup +32) (sup -61) eV and could originate in an accretion disk (with inclination angle greater than approximately 30 deg) around the putative central black hole. The narrow Fe K alpha line has an equivalent width of 1632(sup +47) (sup -26) eV and is unresolved (FWHM < 4630 km per s) and likely originates in distant matter. The absolute flux in the narrow line implies that the column density out of the line-of-sight could be much higher than measured in the line-of-sight, and that the mean (historically-averaged) continuum luminosity responsible for forming the line could be a factor of several higher than that measured from the data. We also detect the Fe K Beta line (corresponding to the narrow Fe K alpha line) with a high signal-to-noise ratio and describe a new robust method to constrain the ionization state of Fe responsible for the Fe K alpha and Fe K Beta lines that does not require any knowledge of possible gravitational and Doppler energy shifts affecting the line energies. For the distant line-emitting matter (e. g. the putative obscuring torus) we deduce that the predominant ionization state is lower than Fe VIII (at 99% confidence), conservatively taking into account residual calibration uncertainties in the XIS energy scale and theoretical and experimental uncertainties in the Fe K fluorescent line energies. From the limits on a possible Compton-reflection continuum it is likely that the narrow Fe K alpha and Fe K Beta lines originate in a Compton-thin structure.
- Published
- 2006
237. Simvastatin treatment for inflammatory attacks of the hyperimmunoglobulinemia D and periodic fever syndrome
- Author
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Simon, Anna, Drewe, Elizabeth, van der Meer, Jos W. M., Powell, Richard J., Kelley, Richard I., Stalenhoef, Anton F. H., and Drenth, Joost P. H.
- Published
- 2004
238. Expression of Msl-2 causes assembly of dosage compensation regulators on the X chromosomes and female lethality in Drosophila
- Author
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Kelley, Richard L., Solovyeva, Irina, Lyman, Laura M., Richman, Ron, Solovyev, Victor, and Kuroda, Mitzi I.
- Subjects
Drosophila -- Physiological aspects ,Dosage compensation (Genetics) -- Research ,Lethal mutation -- Research ,Biological sciences - Abstract
The ectopic expression of the male-specific lethal-2 (msl-2) gene in females causes an increase in the dosage compensation regulators, msl-1 and maleless, on the X chromosomes which decreases the viability and reproductive ability of the female Drosophila. The msl-2 contains many sex lethal protein binding sites and it is male specific although it is present in both males and females. The amount of the protein encoded by msl-2 is more in males than females.
- Published
- 1995
239. Favorable Preliminary Experience With Etanercept in Two Patients With the Hyperimmunoglobulinemia D and Periodic Fever Syndrome
- Author
-
Takada, Kazuki, Aksentijevich, Ivona, Mahadevan, Vijayabhanu, Dean, Jane A., Kelley, Richard I., and Kastner, Daniel L.
- Published
- 2003
240. Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency
- Author
-
Krakowiak, Patrycja A., Wassif, Christopher A., Kratz, Lisa, Cozma, Diana, Kovárová, Martina, Harris, Ginny, Grinberg, Alexander, Yang, Yinzi, Hunter, Alasdair G.W., Tsokos, Maria, Kelley, Richard I., and Porter, Forbes D.
- Published
- 2003
241. Pre- and post-launch operation of the soft x-ray spectrometer Resolve on board the XRISM satellite
- Author
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den Herder, Jan-Willem A., Nikzad, Shouleh, Nakazawa, Kazuhiro, Sato, Kosuke, Ishisaki, Yoshitaka, Kelley, Richard L., Awaki, Hisamitsu, Balleza, Jesus C., Barnstable, Kim R., Bialas, Thomas G., Boissay-Malaquin, Rozenn, Brown, Gregory V., Canavan, Edgar R., Cumbee, Renata S., Carnahan, Timothy M., Chiao, Meng P., Comber, Brian J., Costantini, Elisa, den Herder, Jan-Willem, Dercksen, Johannes, de Vries, Cor, DiPirro, Michael J., Eckart, Megan E., Ezoe, Yuichiro, Ferrigno, Carlo, Fujimoto, Ryuichi, Gorter, Nathalie, Graham, Steven M., Grim, Martin, Hartz, Leslie S., Hayakawa, Ryota, Hayashi, Takayuki, Hell, Natalie, Hoshino, Akio, Ichinohe, Yuto, Ishi, Daiki, Ishida, Manabu, Ishikawa, Kumi, James, Bryan L., Kanemaru, Yoshiaki, Kenyon, Steven J., Kilbourne, Caroline A., Kimball, Mark O., Kitamoto, Shunji, Leutenegger, Maurice A., Maeda, Yoshitomo, McCammon, Dan, McLaughlin, Brian J., Miko, Joseph J., van der Meer, Erik, Mizumoto, Misaki, Okajima, Takashi, Okamoto, Atsushi, Paltani, Stéphane, Porter, Frederick S., Reichenthal, Lillian S., Sato, Toshiki, Sato, Yoichi, Sawada, Makoto, Shinozaki, Keisuke, Shipman, Russell, Shirron, Peter J., Sneiderman, Gary A., Soong, Yang, Szymkiewicz, Richard, Szymkowiak, Andrew E., Takei, Yoh, Takeo, Mai, Tamba, Tsubasa, Tamura, Keisuke, Tsujimoto, Masahiro, Uchida, Yuusuke, Wasserzug, Stephen, Witthoeft, Michael C., Wolfs, Rob, Yamada, Shinya, Yasuda, Susumu, and Yoshida, Seiji
- Published
- 2024
- Full Text
- View/download PDF
242. On-orbit performance of the Adiabatic Demagnetization Refrigerator on XRISM
- Author
-
den Herder, Jan-Willem A., Nikzad, Shouleh, Nakazawa, Kazuhiro, Shirron, Peter J., Kimball, Mark O., Ottens, Richard S., James, Bryan L., Canavan, Edgar R., DiPirro, Michael J., Bialas, Thomas G., Sneiderman, Gary A., Kilbourne, Caroline A., Porter, Frederick S., Kelley, Richard L., Barnstable, Kim R., Fujimoto, Ryuichi, Takei, Yoh, and Yoshida, Seiji
- Published
- 2024
- Full Text
- View/download PDF
243. The Astro-E2 Mission
- Author
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Kelley, Richard L
- Subjects
Astronomy - Abstract
The Astro-E2 observatory is a rebuild of the original Astro-E observatory that was lost during launch in February 2000. It is scheduled for launch into low earth orbit on a Japanese M-V rocket in early 2005. The Institute of Space and Astronautical Science, Japan Aerospace Exploration Agency, is developing the observatory with major contributions from the US. The three instruments on the observatory are the high-resolution x-ray spectrometer (the XRS) featuring a 30-pixel x-ray microcalorimeter array, a set of four CCD cameras (the XIS) and a combination photo-diode/scintillator detector system (the HXD) that will extend the band pass up to nearly 700 keV. A significant feature of Astro-E2 is that all of the instruments are coaligned and operated simultaneously. With its high spectral resolution and collecting area for spectroscopy above 1 keV, Astro-E2 should enable major discovery space and pioneer new technology for use in space. Prime areas for investigation are supernova remnants, active galaxies and the measurement of black hole properties via relativistically-broadened Fe-K emission galaxies. A number of enhancements have been made for the Astro-E2/XRS, including a higher resolution microcalorimeter array, ii mechanical cooler for longer cryogen life, and an improved in-flight calibration system. The Astro-E2/XIS has also been improved to include two back-side-illuminated CCDs to enhance the low energy response. Improvements have also been made to the x-ray mirrors used for both the XRS and XIS to sharpen the point spread function and reduce the effects of stray light. In this talk we will present the essential features of Astro-E2, paying particular attention to the enhancements, and describe the major scientific strengths of the observatory.
- Published
- 2004
244. Hereditary Periodic Fever
- Author
-
Kelley, Richard I., Takada, Kazuki, and Aksentijevich, Ivona
- Published
- 2002
245. Database for Regional Geology, Phase 1: A Tool for Informing Regional Evaluations of Alternative Geologic Media and Decision Making
- Author
-
Perry, Frank Vinton, primary, Kelley, Richard E., additional, Birdsell, Suzanne M., additional, Lugo, Alexander Bryan, additional, Dobson, Patrick, additional, and Houseworth, James, additional
- Published
- 2014
- Full Text
- View/download PDF
246. Phospholipid abnormalities in children with Barth syndrome
- Author
-
Schlame, Michael, Kelley, Richard I, Feigenbaum, Annette, Towbin, Jeffrey A, Heerdt, Paul M, Schieble, Thomas, Wanders, Ronald J.A, DiMauro, Salvatore, and Blanck, Thomas J.J
- Published
- 2003
- Full Text
- View/download PDF
247. Barth syndrome
- Author
-
Clarke Sarah LN, Bowron Ann, Gonzalez Iris L, Groves Sarah J, Newbury-Ecob Ruth, Clayton Nicol, Martin Robin P, Tsai-Goodman Beverly, Garratt Vanessa, Ashworth Michael, Bowen Valerie M, McCurdy Katherine R, Damin Michaela K, Spencer Carolyn T, Toth Matthew J, Kelley Richard I, and Steward Colin G
- Subjects
Barth syndrome ,3-methylglutaconic aciduria ,Dilated cardiomyopathy ,Stillbirth ,Growth delay ,Endocardial fibroelastosis ,Left ventricular non-compaction ,Arrhythmia ,Myopathy ,Neutropenia ,Medicine - Abstract
Abstract First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA). Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), endocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical), compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ) gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and recurrent male miscarriage and stillbirth. Cardiolipin testing and TAZ sequencing now provide relatively rapid diagnostic testing, both prospectively and retrospectively, from a range of fresh or stored tissues, blood or neonatal bloodspots. TAZ sequencing also allows female carrier detection and antenatal screening. Management of BTHS includes medical therapy of CM, cardiac transplantation (in 14% of patients), antibiotic prophylaxis and granulocyte colony-stimulating factor (G-CSF) therapy. Multidisciplinary teams/clinics are essential for minimising hospital attendances and allowing many more individuals with BTHS to live into adulthood.
- Published
- 2013
- Full Text
- View/download PDF
248. X Chromosome Dosage Compensation in Drosophila
- Author
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Birchler, James A., Kelley, Richard L., and Kuroda, Mitzi I.
- Published
- 1996
249. The Smith-Lemli-Opitz syndrome
- Author
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Kelley, Richard I and Hennekam, Raoul C M
- Published
- 2000
250. In-flight Calibration of Hitomi Soft X-ray Spectrometer (3) Effective Area
- Author
-
Eckart, Megan E., Kelley, Richard L., Kilbourne, Caroline A., Leutenegger, Maurice A., Porter, Frederick S., Serlemitsos, Peter J., Szymkowiak, Andrew, Yaqoob, Tahir, Tsujimoto, Masahiro, Okajima, Takashi, Hayashi, Takayuki, Hoshino, Akio, Iizuka, Ryo, Maeda, Yoshitomo, Mori, Hideyuki, Sato, Kosuke, and Sato, Toshiki
- Subjects
Physics ,High Energy Astrophysical Phenomena (astro-ph.HE) ,Spectrometer ,Astrophysics::High Energy Astrophysical Phenomena ,Antenna aperture ,Continuum (design consultancy) ,Flux ,FOS: Physical sciences ,Astronomy and Astrophysics ,01 natural sciences ,Computational physics ,010309 optics ,Crab Nebula ,Space and Planetary Science ,0103 physical sciences ,Calibration ,Satellite ,methods: observational ,space vehicles: instruments ,Astrophysics - Instrumentation and Methods for Astrophysics ,Astrophysics - High Energy Astrophysical Phenomena ,010303 astronomy & astrophysics ,Event (particle physics) ,Instrumentation and Methods for Astrophysics (astro-ph.IM) ,instrumentation: spectrographs - Abstract
著者人数: 17名, Accepted: 2018-01-05, 資料番号: SA1170317000
- Published
- 2018
- Full Text
- View/download PDF
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