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Göbel, Kathrin ; Adrich, Przemysław ; Altstadt, Sebastian ; Altstadt, Sebastian ; Alvarez-Pol, Héctor ; Aksouh, Farouk ; Aumann, Thomas ; Babilon, Mari ; Behr, Karl-Heinz ; Berg, Thomas ; Böhmer, Michael ; Boretzky, Konstanze ; Brünle, Adolf ; Beyer, R. ; Casarejos Ruiz, Enrique ; Chartier, Marielle ; Cortina Gil, Dolores ; Chatillon, Audrey ; Datta Pramanik, Ushasi ; Deveaux, L. ; Elvers, Michael ; Elze, Thomas W. ; Emling, Hans ; Erhard, Martin Andreas ; Ershova, Olga ; Fernández Domínguez, Beatriz ; Geissel, Hans ; Górska, Magdalena ; Heftrich, Tanja ; Heil, Michael ; Hellstroem, M. ; Ickert, Günter ; Johansson, Håkan T. ; Junghans, Arnd Rudolf ; Käppeler, Franz ; Kiselev, Oleg A. ; Klimkiewicz, Adam ; Kratz, Jens-Volker ; Kratz, Jens-Volker ; Kulessa, Reinhard ; Kurz, Nikolaus ; Labiche, Marc ; Langer, Christoph ; Le Bleis, Tudi ; Lemmon, Roy Crawford ; Lindenberg, Kai ; Litvinov, Yuri A. ; Maierbeck, Peter ; Movsesyan, Alina ; Müller, S. ; Nilsson, Thomas ; Nociforo, Chiara ; Paar, Nils ; Palit, Rudrayoti ; Paschalis, Stefanos ; Plag, Ralf ; Prokopowicz, Wawrzyniec ; Reifarth, René ; Rossi, Dominic M. ; Schnorrenberger, Linda ; Simon, Haik ; Sonnabend, Kerstin ; Sümmerer, Klaus ; Surówka, Grzegorz ; Vretenar, Dario ; Wagner, Andreas ; Walter, S. ; Waluś, Władysław ; Wamers, Felix ; Weick, Helmut ; Weigand, Mario ; Winckler, Nicolas ; Winkler, Martin ; Zilges, Andreas
The Coulomb Dissociation (CD) cross sections of the stable isotopes 92,94,100Mo and of the unstable isotope 93Mo were measured at the LAND/R3B setup at GSI Helmholtzzentrum für Schwerionenforschung in Darmstadt, Germany. Experimental data on these isotopes may help to explain the problem of the underproduction of 92,94Mo and 96,98Ru in the models of p-process nucleosynthesis. The CD cross sections obtained for the stable Mo isotopes are in good agreement with experiments performed with real photons, thus validating the method of Coulomb Dissociation. The result for the reaction 93Mo(γ,n) is especially important since the corresponding cross section has not been measured before. A preliminary integral Coulomb Dissociation cross section of the 94Mo(γ,n) reaction is presented. Further analysis will complete the experimental database for the (γ,n) production chain of the p-isotopes of molybdenum.
Barucca, Gianni ; Davì, F. ; Lancioni, G. ; Mengucci, P. ; Montalto, L. ; Natali, P. P. ; Paone, N. ; Rinaldi, D. ; Scalise, L. ; Erni, W. ; Krusche, B. ; Steinacher, M. ; Walford, N. ; Cao, N. ; Liu, Z. ; Liu, C. ; Liu, B. ; Shen, X. ; Sun, S. ; Tao, J. ; Xiong, X. A. ; Zhao, G. ; Zhao, J. ; Albrecht, Malte ; Alkakhi, W. ; Bökelmann, S. ; Coen, S. ; Feldbauer, Florian ; Fink, M. ; Frech, J. ; Freudenreich, V. ; Fritsch, Miriam ; Grochowski, J. ; Hagdorn, R. ; Heinsius, Fritz-Herbert ; Held, T. ; Holtmann, Tobias ; Keshk, Iman ; Koch, H. ; Kopf, Bertram ; Kuhlmann, M. ; Kümmel, M. ; Küßner, M. ; Li, J. ; Linzen, L. ; Maldaner, Stephan ; Oppotsch, J. ; Pankonin, S. ; Pelizäus, Marc ; Pflüger, S. ; Pitka, A. ; Reher, J. ; Reicherz, G. ; Schnier, Claudius ; Steinke, M. ; Triffterer, T. ; Wenzel, C. ; Wiedner, Ulrich ; Denizli, H. ; Er, N. ; Keskin, U. ; Olgun, A. T. ; Yerlikaya, S. ; Yilmaz, A. ; Beck, R. ; Hammann, C. ; Hartmann, J. ; Ketzer, Bernhard ; Müllers, J. ; Rossbach, M. ; Salisbury, B. ; Schmidt, Christian Joachim ; Thoma, U. ; Urban, M. ; Bianconi, A. ; Bragadireanu, Mario ; Pantea, D. ; Domagala, M. ; Filo, G. ; Lisowski, E. ; Lisowski, F. ; Michałek, M. ; Poznański, P. ; Płażek, J. ; Korcyl, K. ; Kozela, A. ; Lebiedowicz, P. ; Pysz, K. ; Schäfer, W. ; Szczurek, A. ; Firlej, M. ; Fiutowski, T. ; Idzik, M. ; Moron, J. ; Swientek, K. ; Terlecki, P. ; Korcyl, G. ; Lalik, R. ; Malige, A. ; Moskal, P. ; Nowakowski, K. ; Przygoda, W. ; Rathod, N. ; Rudy, Z. ; Salabura, P. ; Smyrski, J. ; Augustin, I. ; Böhm, R. ; Lehmann, I. ; Schmitt, L. ; Varentsov, V. ; Al-Turany, Mohammad ; Belias, A. ; Deppe, H. ; Dzhygadlo, R. ; Flemming, H. ; Gerhardt, A. ; Götzen, Klaus ; Heinz, Andreas ; Jiang, P. ; Karabowicz, R. ; Koch, S. ; Kurilla, U. ; Lehmann, D. ; Lühning, J. ; Lynen, U. ; Orth, H. ; Peters, Klaus ; Saito, T. ; Schepers, G. ; Schmidt, C. J. ; Schwarz, C. ; Schwiening, J. ; Täschner, Alexander ; Traxler, M. ; Voss, B. ; Wieczorek, P. ; Abazov, V. ; Alexeev, G. ; Arefiev, V. A. ; Astakhov, V. ; Barabanov, M. Yu. ; Batyunya, B. V. ; Dodokhov, V. Kh. ; Efremov, A. ; Fechtchenko, A. ; Galoyan, A. ; Golovanov, G. ; Koshurnikov, E. K. ; Lobanov, Y. Yu. ; Olshevskiy, A. G. ; Piskun, A. A. ; Samartsev, A. ; Shimanski, S. ; Skachkov, N. B. ; Skachkova, A. N. ; Strokovsky, E. A. ; Tokmenin, V. ; Uzhinsky, V. ; Verkheev, A. ; Vodopianov, A. ; Zhuravlev, N. I. ; Branford, D. ; Watts, D. ; Böhm, M. ; Eyrich, W. ; Lehmann, A. ; Miehling, D. ; Pfaffinger, M. ; Quin, N. ; Robison, L. ; Seth, K. ; Xiao, T. ; Bettoni, Diego ; Ali, A. ; Hamdi, A. ; Himmelreich, Mathilde ; Krebs, M. ; Nakhoul, S. ; Nerling, Frank ; Belousov, A. ; Kisel, Ivan ; Kozlov, Grigory ; Pugach, M. ; Zyzak, Maksym ; Bianchi, Nicola ; Gianotti, Paola ; Lucherini, V. ; Bracco, G. ; Bettner, Y. ; Bodenschatz, S. ; Brinkmann, K. T. ; Brück, L. ; Diehl, S. ; Dormenev, V. ; Düren, M. ; Erlen, T. ; Föhl, K. ; Hahn, C. ; Hayrapetyan, A. ; Hofmann, J. ; Kegel, S. ; Kesselkaul, M. ; Köseoglu, I. ; Kripko, A. ; Kühn, Wolfgang ; Lange, Jens Sören ; Metag, V. ; Moritz, M. ; Nanova, M. ; Novotny, R. ; Orsich, P. ; Pereira-de-Lira, J. ; Peter, M. ; Sachs, M. ; Schmidt, M. ; Schubert, R. ; Stenzel, H. ; Straube, M. ; Strickert, M. ; Thöring, U. ; Wasem, T. ; Wohlfahrt, B. ; Zaunick, H. G. ; Tomasi-Gustafsson, E. ; Glazier, D. ; Ireland, D. ; Seitz, B. ; Deepak, P. N. ; Kulkarni, A. ; Kappert, R. ; Kavatsyuk, Myroslav O. ; Loehner, H. ; Messchendorp, Johannes Gerhardus ; Rodin, Viktor ; Schakel, P. ; Vejdani, S. ; Dutta, K. ; Kalita, K. ; Huang, G. ; Liu, D. ; Peng, H. ; Qi, H. ; Sun, Y. ; Zhou, X. ; Kunze, M. ; Azizi, K. ; Tavukoglu, Z. ; Derichs, A. ; Dosdall, R. ; Esmail, W. ; Gillitzer, A. ; Goldenbaum, F. ; Grunwald, D. ; Jokhovets, L. ; Kannika, J. ; Kulessa, P. ; Orfanitski, S. ; Pérez Andrade, G. ; Prasuhn, D. ; Prencipe, E. ; Pütz, J. ; Ritman, J. ; Rosenthal, E. ; Schadmand, S. ; Schmitz, R. ; Scholl, A. ; Sefzick, T. ; Serdyuk, V. ; Stockmanns, T. ; Veretennikov, D. ; Wintz, P. ; Wüstner, P. ; Xu, H. ; Zhou, Y. ; Cao, X. ; Hu, Q. ; Li, Z. ; Li, H. ; Liang, Y. ; Ma, X. ; Rigato, V. ; Isaksson, L. ; Achenbach, P. ; Corell, O. ; Denig, Achim ; Distler, M. ; Hoek, M. ; Lauth, W. ; Leithoff, H. H. ; Liu, Z. ; Merkel, H. ; Müller, U. ; Pochodzalla, J. ; Schlimme, S. ; Sfienti, C. ; Thiel, M. ; Zambrana, M. ; Ahmed, Samer ; Bleser, S. ; Bölting, M. ; Capozza, L. ; Dbeyssi, A. ; Ehret, A. ; Klasen, R. ; Kliemt, Ralf ; Maas, F. ; Motzko, C. ; Noll, O. ; Piñeiro, D. Rodríguez ; Schupp, F. ; Steinen, M. ; Wolff, S. ; Zimmermann, I. ; Fedorov, A. ; Kazlou, D. ; Korzhik, M. ; Missevitch, O. ; Balanutsa, P. ; Chernetsky, V. ; Demekhin, A. ; Dolgolenko, A. ; Fedorets, P. ; Gerasimov, A. ; Golubev, A. ; Goryachev, V. ; Kantsyrev, Alexey V. ; Kirin, D. Y. ; Kristi, N. ; Ladygina, E. ; Luschevskaya, E. ; Matveev, V. A. ; Panjushkin, V. ; Stavinskiy, A. V. ; Balashoff, A. ; Boukharov, A. ; Malyshev, O. ; Basant, K. N. ; Kumawat, H. ; Roy, B. ; Saxena, Alok ; Yogesh, S. ; Bonaventura, D. ; Brand, P. ; Fritzsch, C. ; Grieser, S. ; Hargens, C. ; Hergemöller, A. K. ; Hetz, B. ; Hüsken, Nils ; Kellers, J. ; Khoukaz, Alfons ; Mannweiler, C. ; Vestrick, S. ; Bumrungkoh, D. ; Herold, Christoph ; Khosonthongkee, K. ; Kobdaj, Chinorat ; Limphirat, A. ; Manasatitpong, K. ; Nasawad, T. ; Pongampai, S. ; Simantathammakul, T. ; Srisawad, P. ; Wongprachanukul, N. ; Yan, Y. ; Yu, C. ; Zhang, X. ; Zhu, W. ; Blinov, A. E. ; Kononov, S. ; Kravchenko, E. A. ; Antokhin, E. ; Barnyakov, A. Yu. ; Beloborodov, K. ; Blinov, V. E. ; Kuyanov, I. A. ; Pivovarov, S. ; Pyata, E. ; Tikhonov, Y. ; Hunter, G. ; Lattery, M. ; Pace, H. ; Boca, Gianluigi ; Duda, D. ; Finger, M. ; Finger, M. ; Kveton, A. ; Pesek, M. ; Peskova, M. ; Prochazka, I. ; Slunecka, M. ; Volf, M. ; Gallus, P. ; Jary, V. ; Korchak, O. ; Marcisovsky, M. ; Neue, G. ; Novy, J. ; Tomasek, L. ; Tomasek, M. ; Virius, M. ; Vrba, V. ; Abramov, V. ; Bukreeva, S. ; Chernichenko, S. ; Derevschikov, A. ; Ferapontov, V. ; Goncharenko, Y. ; Levin, A. ; Maslova, E. ; Melnik, Y. ; Meščanin, Aleksei P. ; Minaev, N. ; Mochalov, V. ; Moiseev, V. ; Morozov, D. ; Nogach, L. ; Poslavskiy, S. ; Ryazantsev, A. ; Ryzhikov, S. ; Semenov, P. ; Shein, I. ; Uzunian, A. ; Vasiliev, Andrey ; Yakutin, A. ; Roy, U. ; Yabsley, B. ; Belostotski, S. ; Fedotov, G. ; Gavrilov, G. ; Izotov, A. ; Manaenkov, S. ; Miklukho, O. ; Zhdanov, A. ; Atac, A. ; Bäck, T. ; Cederwall, B. ; Makonyi, K. ; Preston, M. ; Tegner, P. E. ; Wölbing, D. ; Gandhi, K. ; Rai, A. K. ; Godre, S. ; Crede, V. ; Dobbs, S. ; Eugenio, P. ; Lersch, D. ; Calvo, D. ; De Remigis, P. ; Filippi, A. ; Mazza, G. ; Rivetti, Angelo ; Wheadon, R. ; Bussa, M. P. ; Spataro, Stefano ; Iazzi, F. ; Lavagno, A. ; Martin, A. ; Akram, A. ; Calen, H. ; Ikegami Andersson, Walter ; Johansson, Tord ; Kupsc, Andrzej ; Marciniewski, P. ; Papenbrock, Michael ; Regina, J. ; Rieger, J. ; Schönning, K. ; Wolke, M. ; Pothodi Chackara, V. ; Chlopik, A. ; Kesik, G. ; Melnychuk, D. ; Tarasiuk, J. ; Wojciechowski, M. ; Wronka, S. ; Zwieglinski, B. ; Amsler, C. ; Bühler, Paul ; Marton, J. ; Nalti, W. ; Steinschaden, D. ; Widmann, Eberhard ; Zimmermann, S. ; Zmeskal, Johann
This paper reports on Monte Carlo simulation results for future measurements of the moduli of time-like proton electromagnetic form factors, |GE | and |GM|, using the ¯pp → μ+μ− reaction at PANDA (FAIR). The electromagnetic form factors are fundamental quantities parameterizing the electric and magnetic structure of hadrons. This work estimates the statistical and total accuracy with which the form factors can be measured at PANDA, using an analysis of simulated data within the PandaRoot software framework. The most crucial background channel is ¯pp → π+π−,due to the very similar behavior of muons and pions in the detector. The suppression factors are evaluated for this and all other relevant background channels at different values of antiproton beam momentum. The signal/background separation is based on a multivariate analysis, using the Boosted Decision Trees method. An expected background subtraction is included in this study, based on realistic angular distribuations of the background contribution. Systematic uncertainties are considered and the relative total uncertainties of the form factor measurements are presented.
Reifarth, René ; Altstadt, Sebastian ; Göbel, Kathrin ; Heftrich, Tanja ; Heil, Michael ; Koloczek, Alexander ; Langer, Christoph ; Plag, Ralf ; Pohl, M. ; Sonnabend, Kerstin ; Weigand, Mario ; Adachi, T. ; Aksouh, Farouk ; Al-Khalili, J. ; AlGarawi, M. ; AlGhamdi, S. ; Alkhazov, G. ; Alkhomashi, N. ; Alvarez-Pol, Héctor ; Alvarez-Rodriguez, R. ; Andreev, V. ; Andrei, B. ; Atar, Leyla ; Aumann, Thomas ; Avdeichikov, Vladimir ; Bacri, C. ; Bagchi, S. ; Barbieri, Carlo ; Beceiro, Saul ; Beck, C. ; Beinrucker, Clemens ; Belier, Gilbert ; Bemmerer, Daniel ; Bendel, M. ; Benlliure Anaya, Jose Fernando ; Benzoni, G. ; Berjillos, R. ; Bertini, D. ; Bertulani, Carlos A. ; Bishop, S. ; Blasi, N. ; Bloch, T. ; Blumenfeld, Y. ; Bonaccorso, A. ; Boretzky, Konstanze ; Botvina, Alexander ; Boudard, A. ; Boutachkov, P. ; Boztosun, I. ; Bracco, A. ; Brambilla, S. ; Monago, J.Briz ; Caamano, Manuel ; Caesar, Christoph ; Camera, F. ; Casarejos Ruiz, Enrique ; Catford, Wilton N. ; Cederkäll, Joakim ; Cederwall, B. ; Chartier, Marielle ; Chatillon, Audrey ; Cherciu, M. ; Chulkov, Leonid V. ; Coleman-Smith, P. ; Cortina Gil, Dolores ; Crespi, F. ; Crespo, Raquel ; Cresswell, J. ; Csatlós, Margit ; Déchery, F. ; Davids, B. ; Davinson, Thomas ; Derya, Vera ; Detistov, P. ; Díaz Fernández, Paloma ; DiJulio, D. ; Dmitry, S. ; Doré, D. ; Dueñas, J. ; Dupont, Emmeric ; Egelhof, Peter ; Egorova, I. ; Elekes, Zoltán ; Enders, Joachim ; Endres, Janis ; Ershov, S. ; Ershova, Olga ; Fernández Domínguez, Beatriz ; Fetisov, A. ; Fiori, Enrico ; Fomichev, A. ; Fonseca, M. ; Fraile, L. ; Freer, Martin ; Friese, Jürgen ; Garcia Borge, Maria José ; Galaviz Redondo, Daniel ; Gannon, S. ; Garg, U. ; Gašparić, Igor ; Gasques, L. ; Gastineau, B. ; Geissel, Hans ; Gernhäuser, Roman ; Ghosh, T. ; Gilbert, M. ; Glorius, Jan ; Golubev, Pavel ; Gorshkov, Alexander ; Gourishetty, A. ; Grigorenko, Leonid V. ; Gulyas, J. ; Haiduc, Maria ; Hammache, F. ; Harakeh, Muhsin N. ; Hass, M. ; Heine, Marcel ; Hennig, Andreas ; Henriques, Ana ; Herzberg, Rolf-Dietmar ; Holl, Matthias ; Ignatov, Alexander ; Ignatyuk, A. ; Ilieva, Stoyanka ; Ivanov, Marian ; Iwasa, N. ; Jakobsson, B. ; Johansson, Håkan T. ; Jonson, Björn ; Joshi, P. ; Junghans, Arnd Rudolf ; Jurado Apruzzese, Beatriz ; Körner, G. ; Kalantar, N. ; Kanungo, Rituparna ; Kelic-Heil, Aleksandra ; Kezzar, K. ; Khan, E. ; Khanzadeev, Alexei ; Kiselev, Oleg A. ; Kogimtzis, M. ; Körper, D. ; Kräckmann, S. ; Kröll, Thorsten ; Krücken, Reiner ; Krasznahorkay, Attila ; Kratz, Jens-Volker ; Kresan, Dmytro ; Krings, Thomas ; Krumbholz, A. ; Krupko, S. ; Kulessa, Reinhard ; Kumar, Shyam ; Kurz, Nikolaus ; Kuzmin, E. ; Labiche, Marc ; Langanke, Karlheinz ; Lazarus, I. ; Bleis, T.Le ; Lederer, Claudia ; Lemasson, A. ; Lemmon, Roy Crawford ; Liberati, V. ; Litvinov, Yuri A. ; Löher, Bastian ; Herraiz, J.Lopez ; Münzenberg, G. ; Machado, Jorge ; Maev, E. ; Mahata, Kripamay ; Mancusi, D. ; Marganiec, Justyna ; Perez, M.Martinez ; Marusov, V. ; Mengoni, D. ; Million, B. ; Morcelle, V. ; Moreno, O. ; Movsesyan, Alina ; Nácher González, Enrique ; Najafi, Mohammad Ali ; Nakamura, T. ; Naqvi, F. ; Nikolski, E. ; Nilsson, Thomas ; Nociforo, Chiara ; Nolan, P. ; Novatsky, B. ; Nyman, Göran Hugo ; Ornelas, A. ; Palit, R. ; Pandit, S. ; Panin, Valerii ; Paradela Dobarro, Carlos ; Parkar, V. ; Paschalis, Stefanos ; Pawłowski, P. ; Perea Martinez, Angel ; Pereira, J. ; Petrache, C. ; Petri, Marina ; Pickstone, Simon Glynn ; Pietralla, Norbert ; Pietri, Stephane ; Pivovarov, Y. ; Potlog, P. ; Prokofiev, Andrey ; Rastrepina, G. ; Rauscher, Thomas ; Ribeiro Jimenez, Guillermo ; Ricciardi, Maria Valentina ; Richter, Achim ; Rigollet, Catherine ; Riisager, Karsten ; Rios, A. ; Ritter, C. ; Rodríguez Frutos, Tomás Raúl ; Rodríguez Vignote, Javier ; Röder, Marko ; Romig, Christopher ; Rossi, Dominic M. ; Roussel-Chomaz, P. ; Rout, Prakash Chandra ; Roy, S. ; Söderström, P. ; Saha Sarkar, Maitreyee ; Sakuta, S. ; Salsac, M. ; Sampson, J. ; Sanchez, J. ; Saez, del Rio ; Rosado, J.Sanchez ; Sanjari, Mohammad Shahab ; Sarriguren, P. ; Sauerwein, Anne ; Savran, Deniz ; Scheidenberger, Christoph ; Scheit, Heiko ; Schmidt, Stefan ; Schmitt, C. ; Schnorrenberger, Linda ; Schrock, P. ; Schwengner, R. ; Seddon, D. ; Sherrill, B. ; Shrivastava, A. ; Sidorchuk, S. ; Silva, Joel ; Simon, Haik ; Simpson, E. ; Singh, P. ; Slobodan, D. ; Sohler, D. ; Spieker, Mark-Christoph ; Stach, D. ; Stan, E. ; Stanoiu, M. ; Stepantsov, S. ; Stevenson, P. ; Strieder, F. ; Stuhl, László ; Suda, T. ; Sümmerer, Klaus ; Streicher, Branislav ; Taieb, Julien ; Takechi, M. ; Tanihata, I. ; Taylor, J. ; Tengblad, Olof ; Ter-Akopian, G. ; Terashima, S. ; Teubig, P. ; Thies, Ronja ; Thoennessen, M. ; Thomas, T. ; Thornhill, J. ; Thungstrom, G. ; Timar, J. ; Togano, Yasuhiro ; Tomohiro, U. ; Tornyi, T. ; Tostevin, J. ; Townsley, C. ; Trautmann, Wolfgang ; Trivedi, T. ; Typel, Stefan ; Uberseder, E. ; Udias, J. ; Uesaka, T. ; Uvarov, L. ; Vajta, Z. ; Velho, Paulo ; Vikhrov, V. ; Volknandt, Meiko ; Volkov, Vasily ; Neumann-Cosel, Peter von ; Schmid, Mirko von ; Wagner, A. ; Wamers, Felix ; Weick, Helmut ; Wells, D. ; Westerberg, L. ; Wieland, Oliver ; Wiescher, Michael ; Wimmer, Christine ; Wimmer, K. ; Winfield, John S. ; Winkel, M. ; Woods, Philip John ; Wyss, R. ; Yakorev, Dmitry ; Yavor, M. ; Zamora Cardona, Juan Carlos ; Zartova, I. ; Zerguerras, T. ; Zgura, M. ; Zhdanov, A. ; Zhukov, Mikhail V. ; Zieblinski, M. ; Zilges, Andreas ; Zuber, Kai
The nucleosynthesis of elements beyond iron is dominated by neutron captures in the s and r processes. However, 32 stable, proton-rich isotopes cannot be formed during those processes, because they are shielded from the s-process flow and r-process β-decay chains. These nuclei are attributed to the p and rp process.
For all those processes, current research in nuclear astrophysics addresses the need for more precise reaction data involving radioactive isotopes. Depending on the particular reaction, direct or inverse kinematics, forward or time-reversed direction are investigated to determine or at least to constrain the desired reaction cross sections.
The Facility for Antiproton and Ion Research (FAIR) will offer unique, unprecedented opportunities to investigate many of the important reactions. The high yield of radioactive isotopes, even far away from the valley of stability, allows the investigation of isotopes involved in processes as exotic as the r or rp processes.
An integrative correlation of myopathology, phenotype and genotype in late onset Pompe disease
(2019)
Kulessa, Martin ; Weyer‐Menkhoff, Iris ; Viergutz, Lara ; Kornblum, Cornelia ; Claeys, Kristl G. ; Schneider, Ilka ; Plöckinger, Ursula ; Young, Peter ; Boentert, Matthias ; Vielhaber, Stefan ; Mawrin, Christian ; Bergmann, Markus ; Weis, Joachim ; Ziagaki, Athanasia ; Stenzel, Werner ; Deschauer, Marcus ; Nolte, Dagmar ; Hahn, Andreas ; Schoser, Benedikt ; Schänzer, Anne
Aims: Pompe disease is caused by pathogenic mutations in the alpha 1,4‐glucosidase (GAA) gene and in patients with late onset Pome disease (LOPD), genotype–phenotype correlations are unpredictable. Skeletal muscle pathology includes glycogen accumulation and altered autophagy of various degrees. A correlation of the muscle morphology with clinical features and the genetic background in GAA may contribute to the understanding of the phenotypic variability.
Methods: Muscle biopsies taken before enzyme replacement therapy were analysed from 53 patients with LOPD. On resin sections, glycogen accumulation, fibrosis, autophagic vacuoles and the degree of muscle damage (morphology‐score) were analysed and the results were compared with clinical findings. Additional autophagy markers microtubule‐associated protein 1A/1B‐light chain 3, p62 and Bcl2‐associated athanogene 3 were analysed on cryosections from 22 LOPD biopsies.
Results: The myopathology showed a high variability with, in most patients, a moderate glycogen accumulation and a low morphology‐score. High morphology‐scores were associated with increased fibrosis and autophagy highlighting the role of autophagy in severe stages of skeletal muscle damage. The morphology‐score did not correlate with the patient's age at biopsy, disease duration, nor with the residual GAA enzyme activity or creatine‐kinase levels. In 37 patients with LOPD, genetic analysis identified the most frequent mutation, c.‐32‐13T>G, in 95%, most commonly in combination with c.525delT (19%). No significant correlation was found between the different GAA genotypes and muscle morphology type.
Conclusions: Muscle morphology in LOPD patients shows a high variability with, in most cases, moderate pathology. Increased pathology is associated with more fibrosis and autophagy.
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