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5. Poly(Epsilon-Lysine) Dendrons Inhibit Proliferation in HER2-Overexpressing SKBR3 Breast Cancer Cells at Levels Higher than the Low-Expressing MDA-MB-231 Phenotype and Independently from the Presentation of HER2 Bioligands in Their Structure.

Author

Peregrino, Giordana M. S., Kudsiova, Laila, and Santin, Matteo

Subjects
*HER2 positive breast cancer, *AMINO acid sequence, *DRUG receptors, *SOLID-phase synthesis, *BREAST cancer
Abstract

Among the known breast cancers, the subtype with HER2 receptors-overexpressing cells is associated with a poor prognosis. The adopted monoclonal antibody Trastuzumab has improved clinical outcomes, but it is associated with drug resistance and relatively high costs. The present work adopted the peptide solid-phase synthesis method to synthesise branched poly(ε-lysine) peptide dendrons with 8 branching arms integrating, at their carboxy terminal molecular root, either an arginine or the HER2 receptor-binding sequence LSYCCK or the scramble sequence CSCLYK. These dendrons were synthesised in quantities higher than 100 mg/batch and with a purity exceeding 95%. When tested with two types of breast cancer cells, the dendrons led to levels of inhibition in the HER2 receptor-overexpressing breast cancer cells (SKBR3) comparable to Trastuzumab and higher than breast cancer cells with low receptor expression (MDA-MB-231) where inhibition was more moderate. Noticeably, the presence of the amino acid sequence LSYCCK at the dendron molecular root did not appear to produce any additional inhibitory effect. This was demonstrated also when the scramble sequence CSCLYK was integrated into the dendron and by the lack of any antiproliferative effect by the control linear target sequence. The specific inhibitory effect on proliferation was finally proven by the absence of cytotoxicity and normal expression of the cell migration marker N-Cadherin. Therefore, the present study shows the potential of poly(ε-lysine) dendrons as a cost-effective alternative to Trastuzumab in the treatment of HER2-positive breast cancer. [ABSTRACT FROM AUTHOR]

Published
2024
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15. Abstracts

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Derlon J. M., Petit-taboué M. C., Dauphin F., Courtheoux P., Chapon F., Creissard P., Darcel F., Houtteville J. P., Kaschten, B., Sadzot, B., Stevenaert, A., Tjuvajev, Juri G., Macapinlac, Homer A., Daghighian, Farhad, Ginos, James Z., Finn, Ronald D., Jiaju Zhang, M. S., Beattie, Bradley, Graham, Martin, Larson, Steven M., Blasberg, Ronald G., Levivier, M., Goldman, S., Pirotte, B., Brucher, J. M., Balériaux, D., Luxen, A., Hildebrand, J., Brotchi, J., Go K. G., Kamman R. L., Mooyaart E. L., Heesters M. A. A. M., Sijens, P. E., Oudksrk, M., van Dijk, P., Levendag, P. C., Vecht, Ch. J., Metz, R. J., Kennedy, D. N., Rosen, B. R., Hochberg, F. H., Fishman, A. J., Filipek, P. A., Caviness, V. S., Gross, M. W., Weinzierl, F. X., Trappe, A. E., Goebel, W. E., Frank, A. M., Becker, Georg, Krone, Andreas, Schmidt, Karsten, Hofmann, Erich, Bogdahn, Ulrich, Bencsch, H., Fclber, S., Finkenstedt, G., Kremser, C., Sfockhammer, G., Aichner, F., Bogdahn U., Fröhlich T., Becker G., Krone A., Schlief R., Schürmann J., Jachimczak P., Hofmann E., Roggendorf W., Roosen K., Carapella, C. M., Carpinelli, G., Passalacqua, R., Raus, L., Giannini, M., Mastrostefano, R., Podo, F., Tofani, A., Maslrostefano, R., Mottoles, M., Ferraironi, A., Scelsa, M. G., Oppido, P., Riccio, A., Maini, C. L., Collombier, L., Taillandier, L., Dcbouverie, M., Laurens, M. H., Thouvenot, P., Weber, M., Bertrand, A., Cruickshank G. S., Patterson J., Hadley D., De Witte, Olivier, Hildebrand, Jerzy, Luxen, André, Goldman, Serge, Ernestus, R. -I., Bockhorst, K., Eis, M., Els, T., Hoehn-Berlage, M., Gliese, M., Fründ, R., Geissler, A., Woertgen, C., Holzschuh, M., Goldman, Serge, Levivier, M., Pirotte, B., Brucher, J. M., Luxen, A., Brotchi, J., Hildebrand, J., Hausmann, O., Merlo, A., Jerrnann, E., Uirich, J., Chiquet-Ehrismann, R., Müller, J., Mäcke, H., Gratzl, O., Herholz, K., Ghaemi, M., Würker, M., Pietrzyk, U., Heiss, W. -D., Kotitschke, K., Brandl, M., Tonn, J. C., Haase, A., Bogdahn, U., Kotitschke, K., Muigg, S., Felber, S., Aichner, F., Haase, A., Bogdahn, U., Krone A., Becker G., Woydt M., Roggendorf W., Hofmann E., Bogdahn U., Roosen K., Lanfermann, Heinrich, Heindel, Walter, Kugel, Harald, Erneslus, Ralf -Ingo, Röhn, Gabricle, Lackner, Klaus, Metz, R. J., Kennedy, D. N., Pardo, F. S., Kutke, S., Sorensen, A. G., Hochberg, F. H., Fishman, A. J., Filipek, P. A., Rosen, B. R., Caviness, V. S., Mechtler, L. L., Withiam-Lench, S., Shin, K., Klnkel, W. R., Patel, M., Truax, B., Kinkel, P., Shin, K., Mechtler, L., Ricci M., Pantano P., Maleci A., Pierallini S., Di Stefano D., Bozzao L., Cantore G. P., Röhn, Gabriele, Els, T., Schröder, R., Hoehn-Berlage, M., Ernestus, R. -I., Ruda, R., Mocellini, C., Soffietti, R., Campana, M., Ropolo, R., Riva, A., de Filippi, P. G., Schiffer, D., Salgado D., Rodrigues M., Salgado L., Fonseca A. T., Vieira M. R., Bravo Marques J. M., Satoh, H., Uozumi, T., Kiya, K., Kurisu, K., Arita, K., Sumida, M., Ikawa, F., Tzuk-Shina, Tz., Gomori, J. M., Rubinstein, R., Lossos, A., Siegal, T., Vaalburg, W., Paans, A. M. J., Willemsen, A. T. M., van Waarde, A., Pruim, J., Visser, G. M., Go, K. G., Valentini, S., Ting, Y. L. T., De Rose, R., Chidichimo, G., Corricro, G., van Lcycn-Pilgram, Karin, Erncslus, Ralf -Ingo, Klug, Norfried, van Leyen-Pilgram, K., Ernestus, R. -I., Schröder, R., Klug, N., Woydt M., Krone A., Tonn J. C., Becker G., Neumann U., Roggendorf W., Roosen K., Plate, Karl H., Breier, Georg, Millaucr, Birgit, Weich, Herbert A., Ullrich, Axel, Risau, Werner, Roosen N., Chopra R. K., Mikkelsen T., Rosenblum S. D., Yan P. S., Knight R., Windham J., Rosenblum M. L., Schiffer, D., Attanasio, A., Cavalla, P., Chio, A., Giordana, M. T., Migheli, A., Amberger, V., Hensel, T., Schwab, M. E., Cervoni, Luigi, Celli, Paolo, Tarantino, Roberto, Huettner, C., Tonn, J. C., Berweiler, U., Roggendorf, W., Salmon, I., Rorive, S., Rombaut, K., Pirotte, B., Haot, J., Brotchi, J., Kiss, R., Maugard-Louboutin C., Charrier J., Fayet G., Sagan C., Cuillioere P., Ricolleau G., Martin S., Menegalli-Bogeelli D., Lajat Y., Resche F., Molnàr, Péter, Bárdos, Helga, Ádány, Róza, Rogers, J. P., Pilkington, G. J., Pollo, B., Giaccone, G., Allegranza, A., Bugiani, O., Prim, J., Badia, J., Ribas, E., Coello, F., Shezen, E., Lossos, A., Abramsky, O., Siegal, T., Scerrati M., Roselli R., Iacoangeli M., Pompucci A., Rossi G. F., Deeb, Saleh M. Al., Koreich, Osama, Yaqub, Basim, Moutaery, Khalaf R. Al., Giordana, M. T., Cavalla, P., Chio, A., Marino, S., Vigliani, M. C., Schiffer, D., Deburghgraeve, V., Darcel, F., Gedouin, D., Hassel, M. Ben, Guegan, Y., Jeremic, B., Grujicic, D., Antunovic, V., Matovic, M., Shibamoto, Y., Kallio, Merja, Huhmar, Helena, Kudoh, Ch., Detta, A., Sugiura, K., Hitchcock, E. R., Mastrostefano, R., Di Russo, R., Cipriani§, M., Occhipinti, E. M., Conti, E. M. S., Clowegeser A., Ortler M., Seiwald M., Kostron H., Rajan B., Ross G., Lim C., Ashlcy S., Goode D., Traish D., Brada M., Sanden, G. A. C. vd, Schouten, L. J., Coebergh, J. W. W., Razenberg, P. P. A., Twijnstra, A., Snilders-Keilholz, A., Voormolen, J. H. C., Hermans, J., Leer, J. W. H., Taillandier, L., Baylac, F., Dcbouvcrie, M., Anxionnal, R., Bracard, S., Vignand, J. M., Duprcz, A., Weber, M., Winking, M., Böker, D. K., Simmet, T., Rothbart, David, Strugar, John, Balledux, Jeroen, Criscuolo, Gregory R., Jachimczak, Piotr, Blesch, Armin, Heβdörfer, Birgit, Bogdahn, Ulrich, Ernestus, Ralf -Ingo, Schröder, Roland, Klug, Norfrid, Krouwer, H. G. J., Duinen, S. G. v., Algra, A., Zentner, J., Wolf, H. K., Ostertun, B., Hufnagel, A., Campos, M. G., Solymosi, L., Schramm, J., Newlands, E. S., O'Reilly, S. M., Brampton, M., Soffietti, R., Chio, A., Mocellini, C., Ruda, R., Vigliani, M. C., Schiffer, D., Sciolla, R., Seliak, D., Henriksson, R., Bergenheim, A. T., Björk, P., Gunnarsson, P. -O., Hariz, Ml., Grant, R., Collie, D., Gregor A., Ebmeier K. P., Jarvis G., Lander F., Cull A., Sellar R., Brada, M., Thomas, C., Elyan, S., Hines, F., Ashley, S., Stenning, S., Bernstein J. J., Goldberg W. J., Roelcke U., Von Ammon K., Hausmann O., Radu E. W., Kaech D., Leenders K. L., Fitzek, II, M. M., Aronen J. Efird, Hochberg, F., Gruber, M., Schmidt, E., Rosen, B., Flschman, A., Pardo, P., Afra U. M. U., Sipos, L., Slouik, F., Boiardi A., Salmaggi A., Pozzi A., Farinotti L., Fariselli L., Silvani A., Brandes, A., Scelzi, E., Rigon, A., Zampieri, P., Pignataro, M., Amanzo, P. D'., Amista, P., Rotilio, A., Fiorentino, M. V., Thomas, R., Brazil, L., O'Connor, A. M., Ashley, S., Brada, M., Salvati, Maurizio, Cervoni, Luigi, Puzzilli, Fabrizio, Cervoni, Luigi, Salvati, Maurizio, Raguso, Michele, Cruickshank G. S., Duckworth R., Rumpling R., Rottuci M., Fariselli L., Boiardi A., Broggi G., Plrint, N. G., Sabattini, E., Manetto, V., Gambacorta, H., Poggi, S., Pileri, S., Ferracini, R., Grant, R., Plev D. V., Hopf N. J., Knosp E., Bohl J., Perncczky A., Kiss, R., Salmon, I., Catnby, I., Dewitte, O., Brotchi, J., Pasteels, J. L., Camby, I., Salmon, I., Darro, F., Danguy, A., Brotchi, J., Pasteels, J. L., Kiss, R., Kiu, M. C., Lai, G. M., Yang, T. S., Ng, K. T., Chen, J. S., Chang, C. N., Leung, W. M., Ho, Y. S., Rychter, M. Deblec, Klimek, A., Liberski, P. P., Karpinaka, A., Krauseneck P., Schöffel V., Müller B., Kreth, F. W., Faist, M., Warnke, P. C., Ostertag, C. B., Nielen, K. M. B. v., Visscr, M. C., Lebrun C., Lonjon M., Desjardin T., Michiels J. F., Chanalet Sa. Lagrange J. L., Roche J. L., Chatel M., Mastronardi L., Puzzilli F., Osman Farah J., Lunardi P., Matsutani, M., Ushio, Y., Takakura, K., Menten, Johan, Hamers, Han, Ribot, Jacques, Dom, René, Tcepen, Hans, Müller B., Weidner N., Krauseneck P., Naujocks, G., van Roost, D., Wiestler, O. D., Kuncz, A., Nieder, C., Setzel-Sesterhein, M., Niewald, M., Schnabel, I., O'Neill, K. S., Kitchen, N. D., Wilkins, P. R., Marsh, H. T., Pierce, E., Doshi, R., Deane, R., Previtali, S., Quattrini, A., Nemni, R., Ducati, A., Wrabetz, L., Canal, N., Punt, C. J. A., Stamatakis, L., Giroux, B., Rutten, E., Quigley, Matthew R., Beth Sargent P. A. -C., Flores, Nicholas, Simon, Sheryl, Maroon, Joseph C., Quigley, Matthew R., Beth Sargent P. A. -C., Flores, Nicholas, Maroon, Joseph C., Rocca A. A., Gervasoni C., Castagna A., Picozzi P., Giugni E., Rocca A. A., Tonnarelli G. P., Ducati A., Mangili F., Truci G., Canal N., Giovanelli M., Roelcke U., Von Ammon K., Radu E. W., Leenders K. L., Sachsenheimer, W., Bimmler, T., Seiwald M., Eiter H. Rhomberg W., Ortler M., Obwegesser A., Kostron H., Steilen H., Henn W., Moringlane J. R., Kolles H., Feiden W., Zang K. D., Sleudel W. I., Steinbrecher, Andreas, Schabet, Martin, Heb, Clemens, Bamberg, Michael, Dichgans, Johannes, Stragliotto, G., Delattre, J. Y., Poisson, M., Zampieri, P., Brandes, A., Rigon, A., Tosatto, L., D'Amanzo, P., Menicucci, N., Rotilio, A., Mingrino, S., Steudel, W. I., Feld, R., Henn, W., Zang, K. D., Maire, J. Ph., Caudry, M., Guerin, J., Celerier, D., Salem, N., Demeaux, H., Fahregat, J. F., Kusak, M. E., Bucno, A., Albisua, J., Jerez, P., Sarasa, J. L., Garefa, R., de Campos, J. M., Kusak, M. E., de Campos, J. M., Bueno, A., García-Delgado, R., Sarasa, J. L., García-Sola, R., Lantsov A. A., Shustova T. I., Lcnartz, D., Wellenreuther, R., von Deirnling, A., Köning, W., Menzel, J., Scarpa, S., Manna, A., Reale, M. G., Oppido, P. A., Carapella, C. M., Frati, L., Valery, C. A., Ichen, M., Foncin, J. P., Soubrane, C., Khayat, D., Philippon, J., Vaz, R., Cruz, C., Weis S., Protopapa D., März R., Winkler P. A., Reulen H. J., Bise K., Beuls E., Berg J., Deinsberger, W., Böker, D. K., Samii, M., Caudry, M., Darrouzet, V., Guérin, J., Trouette, R., Causse, N., Bébéar, J. P., Parker, F., Vallee, J. N., Carlier, R., Zerah, M., Lacroix-Jousselin, C., Piepmeier, Joseph M., Kveton, John, Czibulka, Agnes, Tigliev G. S., Chernov M. P., Maslova L. N., Valdueza, José M., Jänisch, Werner, Bock, Alexander, Harms, Lutz, Bessell, E. M., Graus, F., Punt, J., Firth, J., Hope, T., Koriech, Osama, Al Deeb, Saleh, Al Moutaery, Khalaf, Yaqub, B., Silvani A., Salmaggi A., Pozzi A., Franzini A., Boiardi A., Goldbrunner, R., Warmuth-Metz, M., Paulus, W., Tonn, J. -Ch., Roosen, K., Strik I. I., Müller B., Markert C., Pflughaupt K. -W., Krauseneck P., O'Neill, B. P., Dinapoli, R. P., Voges, J., Sturm, V., Deuß, U., Traud, C., Treuer, H., Lehrke, R., Kim, D. G., Müller, R. P., Alexandrov Yu. S., Moutaery, K., Aabed, M., Koreich, O., Ross, G. M., Rajan, B., Traish, D., Ashley, S., Ford, D., Brada, M., Schmeets, I. L. O., Jager, J. J., Pannebakker, M. A. G., de Jong, J. M. A., van Lindert, E., Knosp, E., Kitz, K., Blond, S., Dubois, F., Assaker, R., Baranzelli, M. C., Sleiman, M., Pruvo, J. P., Coche-Dequeant, B., Matsutani M., Takakura K., Sano K., PetriČ-Grabnar, G., Jereb, B., Župančič, N., Koršič, M., Rainov N. G., Burkert W., Ushio, Yukitaka, Kochi, Masato, Itoyama, Youichi, de Campos, J. M., Kusak, M. E., Sarasa, J. L., García, R., Bueno, A., Ferrando, L., Hoang-Xuan, K., Sanson, M., Merel, P., Delattre, J. Y., Poisson, M., Delattre, O., Thomas, G., Hoang-Xuan, K., Delattre, J. Y., Poisson, M., Thomas, G., Haritz, D., Obersen, B., Grochulla, F., Gabel, D., Haselsberger K., Radner H., Pendl G., Brada, M., Laing, R. W., Warrington, A. P., Nowak, P. J. C. M., Kolkman-Deurloo, I. K. K., Visser, A. G., Berge, Hv. d., Niël, C. G. J. H., Levendag, P. C., Bergström P., Hariz M., Löfroth P. -O., Bergenheim T., Henriksson R., Blond, S., Assaker, R., Cortet-rudelli, C., Dewailly, D., Coche-dequeant, B., Castelain, B., Dinapoli, R., Shaw, E., Coffey, R., Earle, J., Foote, R., Schomberg, P., Gorman, D., Girard N., Courel M. N., Delpech B., Haselsberger K., Friehs G. M., Schröttner O., Pendl G., Pötter, R., hawliczek, R., Sperveslage, P., Prott, F. J., Wachter, S., Dieckmann, K., Würker, M., Herholz, K., Pietrzyk, U., Voges, J., Treuer, H., Sturm, V., Bauer, B., Heiss, W. -D., Jund, R., Zimmermann, F., Feldmann, H. J., Gross, M. W., Kneschaurek, P., Molls, M., Lederman, G., Lowry, J., Wertheim, S., Voulsinas, L., Fine, M., Lederman, G., Lowry, J., Wertheim, S., Fine, M., Voutsinas, I., Qian, G., Rashid, H., Lederman, G., Lowry, J., Wertheim, S., Fine, M., Voulsinas, L., Qian, G., Rashid, H., Moutaery, K., Aabed, M., Koreich, O., Scerrati M., Montemaggi P., Iacoangeli M., Pompucci A., Roselli R., Trignani R., Rossi G. F., Shin, K., Mechtler, L., West, C., Grand, W., Shin, K., Sibata, C., West, C., Mechtler, L., Grand, W., Thomas, R., Guerrero, D., James, N., Ashley, S., Gregor, A., Brada, M., Voges, J., Sturm, V., Bramer, R., Pahlke, H., Lehrke, R., Treuer, H., Banik, N., Kim, D. G., Hövels, M., Bernsen H. J. J. A., Rijken P. F. J. W., Van der Sanden B. P. J., Hagemeier N. E. M., Van der Kogel A. J., Koehler P. J., Verbiest H., Jager J., Vecht Ch. J., Ross G. M., McIlwrath A., Brown R., Mottolesb, C., Pierre'Kahn, A., Croux, M., Roche, J. L., Marchai, J., Delhemes, P., Tremoulet, M., Stilhart, B., Chazai, J., Caillaud, P., Ravon, R., Passacha, J., Bouffet, E., Dirven C. M. F., Mooy J. J. A., Molenaar W. M., Lewandowicz, G. M., Grant, N., Harkness, W., Hayward, R., Thomas, D. G. T., Darling, J. L., Delepine, N., Subovici I. I., Cornille B., Markowska S., Alkallaf JC. Desbois, KühI, J., Niethammer, D., Spaar, H. J., Gnekow, A., Havers, W., Berthold, F., Graf, N., Lampert, F., Maass, E., Mertens, R., Schöck, V., Aguzzi, A., Boukhny, A., Smirtukov, S., Prityko, A., Hoiodov, B., Geludkova, O., Nikanorov, A., Levin, P., Rothbart, David, Balledux, Jeroen, Criscuolo, Gregory R., D'haen, B., Van Calenbergh, F., Casaer, P., Dom, R., Menten, J., Goffin, J., Plets, C., Hertel, A., Hernaiz, P., Seipp, C., Siegler, K., Baum, R. P., Maul, F. D., Schwabe, D., Jacobi, G., Kornhuber, B., Hör, G., Menten, J., Casaer, P., Pilkington, G. J., Merzak, A., Rooprai, H. K., Bullock, P., van Domburg P. H. M. F., Wesseling P., Thijssen H. O. M., Wolff, J. E. A., Boos, J., Krähling, K. H., Gressner-Brocks, V., Jürgens, H., Schlegel, J., Scherthan, H., Arens, N., Stumm, Gabi, Kiessling, Marika, Merzak, A., Koochekpour, S., Pilkington, G. J., Reifenberger, G., Reifenberger, J., Liu, L., James, C. D., Wechsler, W., Collins, V. P., Fabel-Schulte, Klaus, Jachimczak, Plotr, Heßdörfer, Birgitt, Baur, Inge, Schlingensiepen, Karl -Hermann, Brysch, Wolgang, Bogdahn, Ulrich, Blesch A., Bosserhoff A. K., Apfel R., Lottspeich F., Jachimczak P., Büttner R., Bogdahn U., Cece, R., Barajon, I., Tazzari, S., Cavaletti, G., Torri-Tarelli, L., Tredici, G., Hecht, B., Turc-Carel, C., Atllas, R., Chatel, M., Gaudray, P., Gioanni, J., Hecht, F., Balledux, Jeroen, Rothbart, David, Criscuolo, Gregory R., de Campos, J. M., Kusak, M. E., Rey, J. A., Bello, M. J., Sarasa, J. L., Dubois, F., Blond, S., Parent, M., Assaker, R., Gosselin, P., Christiaens, J. L., Feld, R., Moringlane, J. R., Steudel, W. I., Schaudies, J. R., Janka M., Tonn J. C., Fischer U., Meese E., Roosen K., Remmelink, M., Salmon, I., Cras, P., Pasteels, J. L., Brotchi, J., Kiss, R., Bensadoun R. J., Frenay M., Formento J. L., Milano G., Lagrange J. L., Grellier P., Lee, J. -Y., Ernestus, R. -I., Riese, H. -H., Cervós-Navarro, J., Reutter, W., Lippitz, B., Scheitinger, C., Scholz, M., Weis, J., Gilsbach, J. M., Füzesi, L., Koochekpour, S., Merzak, A., Pilkington, G. J., Sanson, M., Li, Y. J., Hoang-Xuan, K., Delattre, J. Y., Poisson, M., Hamelin, R., Van de Kelft, Erik, Dams, Erna, Martin, Jean -Jacques, Willems, Patrick, Lehrke R., Voges J., Treuer H., Erdmann J., Müller R. P., Sturm V., Wurm R. E., Warrington A. P., Laing R. W., Sardell S., Hines F., Graham J. D., Brada M., Ushio, Yukitaka, Kuratsu, Jun -ichi, Kochi, Masato, Kitz K., Aichholzer M., Rössler K., Alesch F., Ertl A., Sorensen, P. S., Helweg-Larsen, S., Mourldsen, H., Hansen, H. H., El Sharoum, S. Y., Berfelo, M. W., Theunissen, P. H. M. H., Jager, J. J., de Jong, J. M. A., Fedorcsák, I., Nyáry, I., Osztie, É., Horvath, Á., Kontra, G., Frenay M., Burgoni-chuzel J., Paquis P., Lagrange J. L., Helweg-Larsen, S., Hansen, SW., Sørensen, PS., Salmon, I., Kiss, R., Krauseneck P., Müller B., Morche M., Tonn J. C., Lagerwaard, F. J., Levendag, P. C., Eijkenboom, W. M. H., Schmilz, P. I. M., Lentzsch S., Weber F., Franke J., Dörken B., Lunardi P., Schettini G., Osman Farah J., Qasho R., Mocellini, C., Ruda, R., Soffietti, R., Garabello, D., Sales, S., De Lucchi, R., Vasario, E., Schiffer, D., Muracciole, X., Régis, J., Manera, L., Peragut, J. C., Juin, P., Sedan, R., Nieder, C., Niewald, M., Walter, K., Schnabel, K., Nieder, C., Niewald, N., Nestle, U., Schnabel, K., Berberich, W., Oschmann, P., Theißen R. D., Reuner K. H., Kaps M., Dorndorf W., Martin, K. K., Akinwunmi, J., Rooprai, H. K., Kennedy, A., Linke, A., Ognjenovic, N., Pilkington, G. J., Svadovsky A. I., Peresedov V. V., Bulakov A. A., Butyalko M. Y., Zhirnova I. G., Labunsky D. A., Gnazdizky V. V., Gannushkina I. V., Taphoorn, M. J. B., Potman, R., Barkhof, F., Weerts, J. G., Karim, A. B. M. F., Heimans, J. 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D., Colombo, Bruno M., DiDonato, Stefano, Finocchiaro, Gaetano, Hebeda, K. M., Sterenborg, H. J. C. M., Saarnak, A. E., Wolbers, J. G., van Gemert, M. J. C., Kaaijk P., Troost D., Leenstra S., Das P. K., Bosch D. A., Kostron H., Hochleitner B. W., Obwegeser A., Ortler M., Seiwald M., Vooys, W., Krouwer, H. G. J., de Gast, G. C., Marx, J. J. M., Osman Farah J., Lunardi P., Puzzilli F., Menovsky, T., Beek, J. F., Wolbers, J. G., van Gemert, M. J. C., Naujocks, G., Wiestler, O. D., Schirrmacher, V., Schramm, J., Schmitz, A., Eis-Hübinger, A. M., Piepmeier, p. h., Pedersen, Patricia, Greer, Charles, Quigley, Matthew R., Shih, Tommy, Elrifal, Amr, Rothfus, William, Maroon, Joseph C., Rohertson, L., Rampling, R., Whoteley, T. L., Piumb, J. A., Kerr, D. J., Falina, P. A., Crossan, I. M., Roosen N., Rock J. P., Feng J., Zeng H., Ho K. L., Fenstermacher J. D., Rosenblum M. L., Ruchoux, M. M., Vincent, S., Jonca, F., Plouet, J., Lecomte, M., Samid, D., Thibault, A., Ram, Z., Oldfield, E. H., Myers, C. E., Reed, E., Schabet, M., Herrlinger, U., Buchholz, R., Shoshan, Y., Siegal, T., Siegal, T., Shezen, E., Siegal, Tz., Stockhammer, G., Rosenblum, M., Samid, D., Lieberman, F., Terzis, A. J. A., Bjerkvig, R., Laerum, O. D., Arnold, H., Thibault, A., Samid, D., Figg, W. D., Myers, C. E., Reed, E., Thomas, R., Flux, G., Chittenden, S., Doshi, P., Brazil, L., Thomas, D. G. T., Bignor, D., Zalutsky, M., Brada, M., Tjuvajev, Juri, Kaplitt, Michael, Desai, Revathi, Bradley, M. S., Bettie B. S., Gansbacher, Bernd, Blasberg, Ronald, Haugland, H. K., Saraste, J., Rooseni, K., Laerum, O. D., Vincent, A. J. P. E., Avezaat, C. J. J., Bout, A., Noteboom, J. L., Vecht, C. h., Valerio, D., Hoogerbrugge, P. M., Weber, F., Reszka, R., Zhu, J., Walther, W., List, J., Schulz, W., Wolbers, J. G., Sterenborg, I. I. J. C. M., Kamphorst, W., van Gemert, M. J. C., van Alplien, H. A. M., Salander P., Bergenheim T., Henriksson R., Grant, R., Brazil, L., Thomas, R., Guerrero, D., Laing, R., Ashley, S., Brada, M., Schmidt B., Bauer B., Grau G., Bohnstedt, T., Frydrych A., Franz K., Lorenz R., Brandes, A., Amanzo, P. D'., Zampieri, P., Rigon, A., Scelzi, E., Rotilio, A., Berti, F., Paccagnella, A., Fiorentino, M. V., Müller B., Krauseneck P., van Deventer, P. L., Dellemijn, P. L. I., van den Bent, M. J., Vecht, Ch. J., Kansen, P. J., Tredici, G., Petruccioli, N. G., Cavaletti, G., Cavalletti, E., Kiburg, B., Müller, L. J., Moorer-van Delft, C. M., Heimans, J. J., Boer, H. H., Pace A., Bove L., Pietrangeli A., Innocenti P., Aloe A., Nardi M., Jandolo B., Kellie S. J., De Graaf S. S. N., Bloemhof H., Roebuck D., Dalla Pozza L., Uges D. D. R., Johnston I., Besser M., Chaseling R. A., Koeppen, S., Gründemann, S., Lossos, A., Siegal, T., Nitschke M., Vieregge P., Reusche E., Rob P., Kömpf D., Postma, T. J., Vermorken, J. B., Heimans, J. J., Rampling R. P., Dunlop D. J., Steward M. S., Campbell S. M., Roy S., Hilkens, P. H. E., Verweij, J., van Putten, W. L. J., Vecht, Ch. J., van den Bent, M. J., Hilkens, P. H. E., Moll, J. W. B., van der Burg, M. E. L., Planting, A. S. T., van Putten, W. L. J., Vecht, Ch. J., van den Bent, M. J., Wondrusch E., Zifko U., Drlicek M., Liszka U., Grisold W., Zifko U., Fazeny B., Dittrich Ch., Wondrusch E., Grisold W., Verschuuren, Jan J., Meneses, Patricio I., Rosenfeld, Myrna R., Kaplitt, Michael G., Posner, Jerome B., Dalmau, Josep, Sillevis Smitt P. A. E., Manley G., Posner J. B., Cavaletti, G., Bogliun, G., Margorati, L., Bianchi, G., Drlicek, M., Liska, U., Casati, B., Kolig, C., Grisold, H., Graus, F., Reñe, R., Uchuya, M., Valldeoriola, F., Delattre, J. Y., Benedetti de Cosentiro C., Ortale D., Martinez R., Lambre J., Cagnolati S., Vinai C., Salmaggi A., Nemni R., Silvani A., Forno M. G., Luksch R., Confalonieri P., Boiardi A., Nitschke M., Scholz J., Vieregge P., Kömpf D., Hochberg F. H., Pfeiffer, G., Netzer, J., Hansen, Ch., Eggers, Ch., Hagel Ch., Kunze, K., Verschuuren, Jan J., Rosenblum, Marc K., Lieberman, Frank S., Posner, Jerome B., and Dalmau, Josep

Published
1994
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17. Differential effects of three interferon betas on neutralising antibodies in patients with multiple sclerosis: a follow up study in an independent laboratory. (Paper)

Author

Bertolotto, A., Malucchi, S., Sala, A., Orefice, G., Carrieri, P. B., Capobianco, M., Milano, E., Melis, F., and Giordana, M. T.

Subjects
Interferon beta -- Physiological aspects, Multiple sclerosis -- Physiological aspects, Viral antibodies -- Physiological aspects, Antibodies -- Physiological aspects, Health, Psychology and mental health, Physiological aspects
Abstract

Objective: To evaluate the incidence and the prevalence of neutralising antibodies (NABs) to three interferon beta (IFNβ) products in patients with multiple sclerosis (MS). Methods: Sera were tested from 125 [...]

Published
2002

39. The Italian dementia with Lewy bodies study group (DLB-SINdem): toward a standardization of clinical procedures and multicenter cohort studies design

Author

Bonanni, L, Cagnin, A., Agosta, F., Babiloni, C., Borroni, B., Bozzali, M., Bruni, A. C., Filippi, M., Galimberti, D., Monastero, R., Muscio, C., Parnetti, L., Perani, D., Serra, L., Silani, V., Tiraboschi, P., Padovani, A., On behalf of DLB SINdem study group, Null, Alberici, A., Alberoni, M., Amici, S., Appollonio, I., Arena, M. G., Arighi, A., Avanzi, S., Bagella, C. F., Baglio, F., Barocco, F., Belardinelli, N., Bonuccelli, U., Bottini, G., Bruno Bossio, R., Bruno, G., Buccomino, D., Cacchiò, G., Calabrese, E., Campanelli, A., Canevelli, M., Canu, E. D. G., Cappa, A., Capra, C., Carapelle, E., Caratozzolo, S., Carbone, G. F. S., Cattaruzza, T., Cerami, C., Cester, A., Cheldi, A., Cherchi, R., Chiari, A., Cirafisi, C., Colao, R., Confaloni, A., Conti, M. Z., Costa, A., Costa, B., Cotelli, M. S., Cova, I., Cravello, L., Cumbo, E., Cupidi, C., De Togni, L., Del Din, G., Del Re, M. L., Dentizzi, C., Di Lorenzo, F., Di Stefano, F., Dikova, N., Farina, E., Floris, G., Foti, A., Franceschi, M., Fumagalli, G. G., Gabelli, C., Ghidoni, E., Giannandrea, D., Giordana, M. T., Giorelli, M., Giubilei, F., Grimaldi, L., Grimaldi, R., Guglielmi, V., Lanari, A., Le Pira, F., Letteri, F., Levi Minzi, G. V., Lorusso, S., Ludovico, L., Luzzi, S., Maggiore, L., Magnani, G., Mancini, G., Manconi, F. M., Manfredi, L., Maniscalco, M., Marano, P., Marcon, M., Marcone, A., Marra, C., Martorana, A., Mascia, M. G., Mascia, V., Mauri, M., Mazzei, B., Meloni, M., Merlo, P., Messa, G., Milia, A., Monacelli, F., Montecalvo, G., Moschella, V., Mura, G., Nemni, R., Nobili, F., Notarelli, A., Di Giacomo, R., Onofrj, M., Paci, C., Padiglioni, C., Perini, M., Perotta, D., Perri, Formenti A., Perri, R., Piccininni, C., Piccoli, T., Pilia, G., Pilotto, A., Poli, S., Pomati, S., Pompanin, S., Pucci, E., Puccio, G., Quaranta, D., Rainero, I., Rea, G., Realmuto, S., Riva, M., Rizzetti, M. C., Rolma, G., Rozzini, L., Sacco, L., Saibene, F. L., Scarpini, E., Sensi, S., Seripa, D., Sinforiani, E., Sorbi, S., Sorrentino, Giuseppe, Spallazzi, M., Stracciari, A., Talarico, G., Tassinari, T., Thomas, A., Tiezzi, A., Tomassini, P. F., Trebbastoni, A., Tremolizzo, L., Tripi, G., Ursini, F., Vaianella, L., Valluzzi, F., Vezzadini, G., Vista, M., Volontè, M. A., Bonanni, L, Cagnin, A, Agosta, F, Babiloni, C, Borroni, B, Bozzali, M, Bruni, A, Filippi, M, Galimberti, D, Monastero, R, Muscio, C, Parnetti, L, Perani, D, Serra, L, Silani, V, Tiraboschi, P, Padovani, A, Alberici, A, Alberoni, M, Amici, S, Appollonio, I, Arena, M, Arighi, A, Avanzi, S, Bagella, C, Baglio, F, Barocco, F, Belardinelli, N, Bonuccelli, U, Bottini, G, Bruno Bossio, R, Bruno, G, Buccomino, D, Cacchiò, G, Calabrese, E, Campanelli, A, Canevelli, M, Canu, E, Cappa, A, Capra, C, Carapelle, E, Caratozzolo, S, Carbone, G, Cattaruzza, T, Cerami, C, Cester, A, Cheldi, A, Cherchi, R, Chiari, A, Cirafisi, C, Colao, R, Confaloni, A, Conti, M, Costa, A, Costa, B, Cotelli, M, Cova, I, Cravello, L, Cumbo, E, Cupidi, C, de Togni, L, Del Din, G, Del Re, M, Dentizzi, C, Di Lorenzo, F, Di Stefano, F, Dikova, N, Farina, E, Floris, G, Foti, A, Franceschi, M, Fumagalli, G, Gabelli, C, Ghidoni, E, Giannandrea, D, Giordana, M, Giorelli, M, Giubilei, F, Grimaldi, L, Grimaldi, R, Guglielmi, V, Lanari, A, Le Pira, F, Letteri, F, Levi Minzi, G, Lorusso, S, Ludovico, L, Luzzi, S, Maggiore, L, Magnani, G, Mancini, G, Manconi, F, Manfredi, L, Maniscalco, M, Marano, P, Marcon, M, Marcone, A, Marra, C, Martorana, A, Mascia, M, Mascia, V, Mauri, M, Mazzei, B, Meloni, M, Merlo, P, Messa, G, Milia, A, Monacelli, F, Montecalvo, G, Moschella, V, Mura, G, Nemni, R, Nobili, F, Notarelli, A, Di Giacomo, R, Onofrj, M, Paci, C, Padiglioni, C, Perini, M, Perotta, D, Perri, F, Perri, R, Piccininni, C, Piccoli, T, Pilia, G, Pilotto, A, Poli, S, Pomati, S, Pompanin, S, Pucci, E, Puccio, G, Quaranta, D, Rainero, I, Rea, G, Realmuto, S, Riva, M, Rizzetti, M, Rolma, G, Rozzini, L, Sacco, L, Saibene, F, Scarpini, E, Sensi, S, Seripa, D, Sinforiani, E, Sorbi, S, Sorrentino, G, Spallazzi, M, Stracciari, A, Talarico, G, Tassinari, T, Thomas, A, Tiezzi, A, Tomassini, P, Trebbastoni, A, Tremolizzo, L, Tripi, G, Ursini, F, Vaianella, L, Valluzzi, F, Vezzadini, G, Vista, M, Volontè, M, Bruni, Ac, DLB-SINdem study, Group, Bruni, AC, and Padovani, A - On behalf of DLB-SINdem study group

Subjects
Lewy Body Disease, medicine.medical_specialty, Pediatrics, Dementia with Lewy bodie, Dementia with Lewy bodies, Dermatology, Cohort Studies, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Quality of life, Alzheimer Disease, Surveys and Questionnaires, mental disorders, Standardization of diagnostic procedures, Diagnosis, Survey, Disease Management, Humans, Italy, Research Design, 2708, Neurology (clinical), Psychiatry and Mental Health, medicine, Dementia, 030212 general & internal medicine, MED/01 - STATISTICA MEDICA, MED/26 - NEUROLOGIA, business.industry, Standardization of diagnostic procedure, General Medicine, medicine.disease, Settore MED/26 - NEUROLOGIA, Cohort, Differential, Physical therapy, Delirium, Alzheimer's disease, medicine.symptom, business, 030217 neurology & neurosurgery, Frontotemporal dementia, Cohort study
Abstract

Dementia with Lewy bodies (DLB) causes elevated outlays for the National Health Systems due to high institutionalization rate and patients' reduced quality of life and high mortality. Furthermore, DLB is often misdiagnosed as Alzheimer's disease. These data motivate harmonized multicenter longitudinal cohort studies to improve clinical management and therapy monitoring. The Italian DLB study group of the Italian Neurological Society for dementia (SINdem) developed and emailed a semi-structured questionnaire to 572 national dementia centers (from primary to tertiary) to prepare an Italian large longitudinal cohort. The questionnaire surveyed: (1) prevalence and incidence of DLB; (2) clinical assessment; (3) relevance and availability of diagnostic tools; (4) pharmacological management of cognitive, motor, and behavioural disturbances; (5) causes of hospitalization, with specific focus on delirium and its treatment. Overall, 135 centers (23.6 %) contributed to the survey. Overall, 5624 patients with DLB are currently followed by the 135 centers in a year (2042 of them are new patients). The percentage of DLB patients was lower (27 ± 8 %) than that of Alzheimer's disease and frontotemporal dementia (56 ± 27 %) patients. The majority of the centers (91 %) considered the clinical and neuropsychological assessments as the most relevant procedure for a DLB diagnosis. Nonetheless, most of the centers has availability of magnetic resonance imaging (MRI; 95 %), electroencephalography (EEG; 93 %), and FP-CIT single photon emission-computerized tomography (SPECT; 75 %) scan for clinical applications. It will be, therefore, possible to recruit a large harmonized Italian cohort of DLB patients for future cross-sectional and longitudinal multicenter studies.

Published
2017

41. Italian Frontotemporal Dementia Network (FTD Group-SINDEM): sharing clinical and diagnostic procedures in Frontotemporal Dementia in Italy

Author

Borroni, B, Turrone, R, Galimberti, D, Nacmias, B, Alberici, A, Benussi, A, Caffarra, P, Caltagirone, C, Cappa, S, Frisoni, G, Ghidoni, R, Marra, C, Padovani, A, Rainero, I, Scarpini, E, Silani, V, Sorbi, S, Tagliavini, F, Tremolizzo, L, Bruni, A, Agosta, F, Alberoni, M, Appollonio, I, Arighi, A, Avanzi, S, Baglio, F, Benussi, L, Bianchetti, A, Binetti, G, Bonanni, L, Bottacchi, E, Bruno, G, Canevelli, M, Canu, E, Cerami, C, Chiari, A, Conti, M, Costa, A, Costa, M, Cotelli, M, Cupidi, C, Daniele, A, D'Anna, S, de Caro, M, De Togni, L, Dell'Osa, M, Di Stefano, F, Ferrarese, C, Ferrari, C, Filastro, F, Floris, G, Franceschi, M, Gennuso, M, Ghidoni, E, Giordana, M, Gragnaniello, D, Grimaldi, L, Lanari, A, Le Pira, F, Lombardi, G, Lorusso, S, Ludovico, L, Luzzi, S, Magnani, G, Manfredi, L, Marano, P, Marcone, A, Marrosu, M, Martorana, A, Mascia, M, Masullo, C, Mauri, M, Mazzone, A, Mela, A, Merlo, P, Micheli, A, Milia, A, Mina, C, Montella, P, Mura, G, Murru, M, Nemni, R, Paci, C, Pantieri, R, Panza, F, Parnetti, L, Perini, M, Pettenati, C, Piccininni, M, Piccoli, T, Pilia, G, Pinessi, L, Piras, M, Realmuto, S, Ricca, I, Rizzetti, M, Rozzini, L, Rubino, E, Sambati, L, Seripa, D, Siano, P, Sinforiani, E, Sorrentino, G, Specchio, L, Stracciari, A, Susani, E, Talarico, G, Tartaglione, B, Tessitore, A, Thomas, A, Tiezzi, A, Tiraboschi, P, Tognoni, G, Tondelli, M, Trebbastoni, A, Turla, M, Ursini, F, Valluzzi, F, Vista, M, Zannino, G, Zanusso, G, Borroni B., Turrone R., Galimberti D., Nacmias B., Alberici A., Benussi A., Caffarra P., Caltagirone C., Cappa S. F., Frisoni G. B., Ghidoni R., Marra C., Padovani A., Rainero I., Scarpini E., Silani V., Sorbi S., Tagliavini F., Tremolizzo L., Bruni A. C., Agosta F., Alberoni M., Appollonio I., Arighi A., Avanzi S., Baglio F., Benussi L., Bianchetti A., Binetti G., Bonanni L., Bottacchi E., Bruno G., Canevelli M., Canu E., Cerami C., Chiari A., Conti M. Z., Costa A., Costa M., Cotelli M., Cotelli M. S., Cupidi C., Daniele A., D'Anna S., de Caro M. F., De Togni L., Dell'Osa M. T., Di Stefano F., Ferrarese C., Ferrari C., Filastro F., Floris G., Franceschi M., Gennuso M., Ghidoni E., Giordana M. T., Gragnaniello D., Grimaldi L., Lanari A., Le Pira F., Lombardi G., Lorusso S., Ludovico L., Luzzi S., Magnani G., Manfredi L. G., Marano P., Marcone A., Marrosu M. G., Martorana A., Mascia M. G., Masullo C., Mauri M., Mazzone A., Mela A., Merlo P., Micheli A., Milia A., Mina C., Montella P., Mura G., Murru M. R., Nemni R., Paci C., Pantieri R., Panza F., Parnetti L., Perini M., Pettenati C., Piccininni M., Piccoli T., Pilia G., Pinessi L., Piras M. R., Realmuto S., Ricca I., Rizzetti M. C., Rozzini L., Rubino E., Sambati L., Seripa D., Siano P., Sinforiani E., Sorrentino G., Specchio L. M., Stracciari A., Susani E., Talarico G., Tartaglione B., Tessitore A., Thomas A., Tiezzi A., Tiraboschi P., Tognoni G., Tondelli M., Trebbastoni A., Turla M., Ursini F., Valluzzi F., Vista M., Zannino G., Zanusso G., Borroni, B, Turrone, R, Galimberti, D, Nacmias, B, Alberici, A, Benussi, A, Caffarra, P, Caltagirone, C, Cappa, S, Frisoni, G, Ghidoni, R, Marra, C, Padovani, A, Rainero, I, Scarpini, E, Silani, V, Sorbi, S, Tagliavini, F, Tremolizzo, L, Bruni, A, Agosta, F, Alberoni, M, Appollonio, I, Arighi, A, Avanzi, S, Baglio, F, Benussi, L, Bianchetti, A, Binetti, G, Bonanni, L, Bottacchi, E, Bruno, G, Canevelli, M, Canu, E, Cerami, C, Chiari, A, Conti, M, Costa, A, Costa, M, Cotelli, M, Cupidi, C, Daniele, A, D'Anna, S, de Caro, M, De Togni, L, Dell'Osa, M, Di Stefano, F, Ferrarese, C, Ferrari, C, Filastro, F, Floris, G, Franceschi, M, Gennuso, M, Ghidoni, E, Giordana, M, Gragnaniello, D, Grimaldi, L, Lanari, A, Le Pira, F, Lombardi, G, Lorusso, S, Ludovico, L, Luzzi, S, Magnani, G, Manfredi, L, Marano, P, Marcone, A, Marrosu, M, Martorana, A, Mascia, M, Masullo, C, Mauri, M, Mazzone, A, Mela, A, Merlo, P, Micheli, A, Milia, A, Mina, C, Montella, P, Mura, G, Murru, M, Nemni, R, Paci, C, Pantieri, R, Panza, F, Parnetti, L, Perini, M, Pettenati, C, Piccininni, M, Piccoli, T, Pilia, G, Pinessi, L, Piras, M, Realmuto, S, Ricca, I, Rizzetti, M, Rozzini, L, Rubino, E, Sambati, L, Seripa, D, Siano, P, Sinforiani, E, Sorrentino, G, Specchio, L, Stracciari, A, Susani, E, Talarico, G, Tartaglione, B, Tessitore, A, Thomas, A, Tiezzi, A, Tiraboschi, P, Tognoni, G, Tondelli, M, Trebbastoni, A, Turla, M, Ursini, F, Valluzzi, F, Vista, M, Zannino, G, Zanusso, G, Borroni B., Turrone R., Galimberti D., Nacmias B., Alberici A., Benussi A., Caffarra P., Caltagirone C., Cappa S. F., Frisoni G. B., Ghidoni R., Marra C., Padovani A., Rainero I., Scarpini E., Silani V., Sorbi S., Tagliavini F., Tremolizzo L., Bruni A. C., Agosta F., Alberoni M., Appollonio I., Arighi A., Avanzi S., Baglio F., Benussi L., Bianchetti A., Binetti G., Bonanni L., Bottacchi E., Bruno G., Canevelli M., Canu E., Cerami C., Chiari A., Conti M. Z., Costa A., Costa M., Cotelli M., Cotelli M. S., Cupidi C., Daniele A., D'Anna S., de Caro M. F., De Togni L., Dell'Osa M. T., Di Stefano F., Ferrarese C., Ferrari C., Filastro F., Floris G., Franceschi M., Gennuso M., Ghidoni E., Giordana M. T., Gragnaniello D., Grimaldi L., Lanari A., Le Pira F., Lombardi G., Lorusso S., Ludovico L., Luzzi S., Magnani G., Manfredi L. G., Marano P., Marcone A., Marrosu M. G., Martorana A., Mascia M. G., Masullo C., Mauri M., Mazzone A., Mela A., Merlo P., Micheli A., Milia A., Mina C., Montella P., Mura G., Murru M. R., Nemni R., Paci C., Pantieri R., Panza F., Parnetti L., Perini M., Pettenati C., Piccininni M., Piccoli T., Pilia G., Pinessi L., Piras M. R., Realmuto S., Ricca I., Rizzetti M. C., Rozzini L., Rubino E., Sambati L., Seripa D., Siano P., Sinforiani E., Sorrentino G., Specchio L. M., Stracciari A., Susani E., Talarico G., Tartaglione B., Tessitore A., Thomas A., Tiezzi A., Tiraboschi P., Tognoni G., Tondelli M., Trebbastoni A., Turla M., Ursini F., Valluzzi F., Vista M., Zannino G., and Zanusso G.

Abstract

In the prospect of improved disease management and future clinical trials in Frontotemporal Dementia, it is desirable to share common diagnostic procedures. To this aim, the Italian FTD Network, under the aegis of the Italian Neurological Society for Dementia, has been established. Currently, 85 Italian Centers involved in dementia care are part of the network. Each Center completed a questionnaire on the local clinical procedures, focused on (1) clinical assessment, (2) use of neuroimaging and genetics; (3) support for patients and caregivers; (4) an opinion about the prevalence of FTD. The analyses of the results documented a comprehensive clinical and instrumental approach to FTD patients and their caregivers in Italy, with about 1,000 newly diagnosed cases per year and 2,500 patients currently followed by the participating Centers. In analogy to other European FTD consortia, future aims will be devoted to collect data on epidemiology of FTD and its subtypes and to provide harmonization of procedures among Centers.

Published
2015

46. Italian Frontotemporal Dementia Network (FTD Group-SINDEM): sharing clinical and diagnostic procedures in Frontotemporal Dementia in Italy

Author

Borroni B., Turrone R., Galimberti D., Nacmias B., Alberici A., Benussi A., Caffarra P., Caltagirone C., Cappa S. F., Frisoni G. B., Ghidoni R., Marra C., Padovani A., Rainero I., Scarpini E., Silani V., Sorbi S., Tagliavini F., Tremolizzo L., Bruni A. C., Agosta F., Alberoni M., Appollonio I., Arighi A., Avanzi S., Baglio F., Benussi L., Bianchetti A., Binetti G., Bonanni L., Bottacchi E., Bruno G., Canevelli M., Canu E., Cerami C., Chiari A., Conti M. Z., Costa A., Costa M., Cotelli M., Cotelli M. S., Cupidi C., Daniele A., D'Anna S., de Caro M. F., De Togni L., Dell'Osa M. T., Di Stefano F., Ferrarese C., Ferrari C., Filastro F., Floris G., Franceschi M., Gennuso M., Ghidoni E., Giordana M. T., Gragnaniello D., Grimaldi L., Lanari A., Le Pira F., Lombardi G., Lorusso S., Ludovico L., Luzzi S., Magnani G., Manfredi L. G., Marano P., Marcone A., Marrosu M. G., Martorana A., Mascia M. G., Masullo C., Mauri M., Mazzone A., Mela A., Merlo P., Micheli A., Milia A., Mina C., Montella P., Mura G., Murru M. R., Nemni R., Paci C., Pantieri R., Panza F., Parnetti L., Perini M., Pettenati C., Piccininni M., Piccoli T., Pilia G., Pinessi L., Piras M. R., Realmuto S., Ricca I., Rizzetti M. C., Rozzini L., Rubino E., Sambati L., Seripa D., Siano P., Sinforiani E., Sorrentino G., Specchio L. M., Stracciari A., Susani E., Talarico G., Tartaglione B., Tessitore A., Thomas A., Tiezzi A., Tiraboschi P., Tognoni G., Tondelli M., Trebbastoni A., Turla M., Ursini F., Valluzzi F., Vista M., Zannino G., Zanusso G., Piccoli, T, B. Borroni, R. Turrone, D. Galimberti, B. Nacmia, A. Alberici, A. Benussi, P. Caffarra, C. Caltagirone, S. F. Cappa, G. B. Frisoni, R. Ghidoni, C. Marra, A. Padovani, I. Rainero, E. Scarpini, V. Silani, S. Sorbi, F. Tagliavini, L. Tremolizzo, A. C. Bruni, The FTD Group-SINDEM, Borroni, B, Turrone, R, Galimberti, D, Nacmias, B, Alberici, A, Benussi, A, Caffarra, P, Caltagirone, C, Cappa, Sf, Frisoni, Gb, Ghidoni, R, Marra, C, Padovani, A, Rainero, I, Scarpini, E, Silani, V, Sorbi, S, Tagliavini, F, Tremolizzo, L, Bruni, Ac, The FTD, Group-SINDEM, Agosta, F, Cappa, S, Frisoni, G, Bruni, A, Alberoni, M, Appollonio, I, Arighi, A, Avanzi, S, Baglio, F, Benussi, L, Bianchetti, A, Binetti, G, Bonanni, L, Bottacchi, E, Bruno, G, Canevelli, M, Canu, E, Cerami, C, Chiari, A, Conti, M, Costa, A, Costa, M, Cotelli, M, Cupidi, C, Daniele, A, D'Anna, S, de Caro, M, De Togni, L, Dell'Osa, M, Di Stefano, F, Ferrarese, C, Ferrari, C, Filastro, F, Floris, G, Franceschi, M, Gennuso, M, Ghidoni, E, Giordana, M, Gragnaniello, D, Grimaldi, L, Lanari, A, Le Pira, F, Lombardi, G, Lorusso, S, Ludovico, L, Luzzi, S, Magnani, G, Manfredi, L, Marano, P, Marcone, A, Marrosu, M, Martorana, A, Mascia, M, Masullo, C, Mauri, M, Mazzone, A, Mela, A, Merlo, P, Micheli, A, Milia, A, Mina, C, Montella, P, Mura, G, Murru, M, Nemni, R, Paci, C, Pantieri, R, Panza, F, Parnetti, L, Perini, M, Pettenati, C, Piccininni, M, Pilia, G, Pinessi, L, Piras, M, Realmuto, S, Ricca, I, Rizzetti, M, Rozzini, L, Rubino, E, Sambati, L, Seripa, D, Siano, P, Sinforiani, E, Sorrentino, G, Specchio, L, Stracciari, A, Susani, E, Talarico, G, Tartaglione, B, Tessitore, A, Thomas, A, Tiezzi, A, Tiraboschi, P, Tognoni, G, Tondelli, M, Trebbastoni, A, Turla, M, Ursini, F, Valluzzi, F, Vista, M, Zannino, G, and Zanusso, G

Subjects
Counseling, Male, medicine.medical_specialty, Neurology, Network, Frontotemporal dementia, Frontotemporal lobar degeneration, Genetics, Survey, Aged, Aged, 80 and over, Caregivers, Female, Frontotemporal Dementia, Humans, Italy, Prevalence, Community Networks, Information Dissemination, Medicine (all), 2708, Neurology (clinical), Psychiatry and Mental Health, Dermatology, ddc:616.89, Caregivers/psychology, Epidemiology, mental disorders, medicine, 80 and over, Dementia, Disease management (health), Psychiatry, MED/26 - NEUROLOGIA, Italian network, FRONTO Temporal dementia, business.industry, Frontotemporal dementia, Frontotemporal lobar degeneration, Network, Survey, Genetics, Counseling, General Medicine, Frontotemporal Dementia/diagnosis/epidemiology, medicine.disease, Clinical trial, Settore MED/26 - Neurologia, Neurosurgery, business
Abstract

In the prospect of improved disease management and future clinical trials in Frontotemporal Dementia, it is desirable to share common diagnostic procedures. To this aim, the Italian FTD Network, under the aegis of the Italian Neurological Society for Dementia, has been established. Currently, 85 Italian Centers involved in dementia care are part of the network. Each Center completed a questionnaire on the local clinical procedures, focused on (1) clinical assessment, (2) use of neuroimaging and genetics; (3) support for patients and caregivers; (4) an opinion about the prevalence of FTD. The analyses of the results documented a comprehensive clinical and instrumental approach to FTD patients and their caregivers in Italy, with about 1,000 newly diagnosed cases per year and 2,500 patients currently followed by the participating Centers. In analogy to other European FTD consortia, future aims will be devoted to collect data on epidemiology of FTD and its subtypes and to provide harmonization of procedures among Centers.

Published
2014

47. FUS/TLS genetic variability in sporadic frontotemporal lobar degeneration

Author

Cantoni, C, Fenoglio, C, Cortini, F, Venturelli, E, Villa, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Franceschi, M, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti, D, Cantoni C., Fenoglio C., Cortini F., Venturelli E., Villa C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Franceschi M., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., Scarpini E., Galimberti D., Cantoni, C, Fenoglio, C, Cortini, F, Venturelli, E, Villa, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Franceschi, M, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti, D, Cantoni C., Fenoglio C., Cortini F., Venturelli E., Villa C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Franceschi M., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., Scarpini E., and Galimberti D.

Abstract

Two hundred and fifty one Italian patients with sporadic frontotemporal lobar degeneration (FTLD) and 259 age-matched controls were tested for association with the tagging single nucleotide polymorphisms (SNPs) rs741810 and rs1052352 in the fused in sarcoma/translated in liposarcoma gene (FUS/TLS). Only patients negative for GRN mutations were included. Considering each SNP alone, no differences in either allelic or genotypic frequencies between patients and controls were found (P > 0.05), even stratifying according to gender or the presence of concomitant motor neuron disease. Haplotype analysis failed to detect haplotypes associated with FTLD. According to these results, FUS/TLS is not a susceptibility factor for the development of sporadic FTLD. © 2010-IOS Press and the authors. All rights reserved.

Published
2010

48. GRN variability contributes to sporadic frontotemporal lobar degeneration

Author

Galimberti, D, Fenoglio, C, Cortini, F, Serpente, M, Venturelli, E, Villa, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Restelli, I, Boneschi, F, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti D., Fenoglio C., Cortini F., Serpente M., Venturelli E., Villa C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Restelli I., Boneschi F. M., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., Scarpini E., Galimberti, D, Fenoglio, C, Cortini, F, Serpente, M, Venturelli, E, Villa, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Restelli, I, Boneschi, F, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti D., Fenoglio C., Cortini F., Serpente M., Venturelli E., Villa C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Restelli I., Boneschi F. M., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., and Scarpini E.

Abstract

Mutations in the progranulin gene (GRN) are responsible for familial FTLD with ubiquitin pathology (FTLD-U). However, there are controversial data regarding the contribution of GRN variability to sporadic FTLD. We carried out an association study in 265 patients, who did not carry a GRN causal mutation, and 375 age-matched controls. Four tagging Single Nucleotide Polymorphisms (SNPs) were chosen generate 80% power to detect an allelic association with P ≤ 0.01. In addition, a known functional SNP (rs5848) was included. An increased frequency of the rs4792938 CC genotype in cases compared with controls was observed (17.4 versus 10.4%, P=0.01, OR: 1.81, 95%CI: 1.15-2.85). Stratifying for gender, no differences were observed for all polymorphisms. Haplotype analysis failed to detect haplotypes associated with the disease. Our findings indicate that the GRN rs4792938 CC genotype represents a susceptibility factor for the development of FTLD in individuals who do not carry GRN causal mutations. This SNP is likely located in a regulatory region, thus an effect on GRN mRNA levels may be of mechanistic importance. © 2010 - IOS Press and the authors. All rights reserved.

Published
2010

49. Is KIF24 a genetic risk factor for Frontotemporal Lobar Degeneration?

Author

Venturelli, E, Villa, C, Fenoglio, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Cortini, F, Fumagalli, G, Cappa, S, Binetti, G, Franceschi, M, Rainero, I, Giordana, M, Mariani, C, Bresolin, N, Scarpini, E, Galimberti, D, Venturelli E., Villa C., Fenoglio C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Cortini F., Fumagalli G., Cappa S., Binetti G., Franceschi M., Rainero I., Giordana M. T., Mariani C., Bresolin N., Scarpini E., Galimberti D., Venturelli, E, Villa, C, Fenoglio, C, Clerici, F, Marcone, A, Benussi, L, Ghidoni, R, Gallone, S, Scalabrini, D, Cortini, F, Fumagalli, G, Cappa, S, Binetti, G, Franceschi, M, Rainero, I, Giordana, M, Mariani, C, Bresolin, N, Scarpini, E, Galimberti, D, Venturelli E., Villa C., Fenoglio C., Clerici F., Marcone A., Benussi L., Ghidoni R., Gallone S., Scalabrini D., Cortini F., Fumagalli G., Cappa S., Binetti G., Franceschi M., Rainero I., Giordana M. T., Mariani C., Bresolin N., Scarpini E., and Galimberti D.

Abstract

Linkage analysis identified a region on chromosome 9p associated with Frontotemporal Lobar Degeneration (FTLD). A detailed analysis of candidate genes lying in this region demonstrated an association with Ubiquitin Associated Protein (UBAP)1. The distribution of five Single Nucleotide Polymorphisms (SNPs) located in the chromosome 9 haplotype identified via linkage analysis, including UBAP1 rs7018487, UBAP2 rs1785506 and rs307658, and KIF24 rs17350674 and rs10814083, has been determined in a population of 284 patients diagnosed with FTLD, including 245 with behavioural variant Frontotemporal Dementia (bvFTD), 23 with Progressive Aphasia and 16 with Semantic Dementia, compared with 318 age-matched controls. A statistically significant increased frequency of the KIF24 rs17350674 AA genotype was observed in patients compared with controls (7.4 versus 2.5%; P= 0.0068, OR: 3.63, CI: 1.58-8.35). Considering each syndrome separately, similar results where obtained in bvFTD versus controls (7.7 versus 2.5%, P= 0.005, OR: 3.26, CI: 1.40-7.57). Stratifying for gender, a statistically significant increased genotypic frequency was observed in female patients as compared with female controls (8.9 versus 2.5%, P= 0.008, OR: 3.85, CI: 1.36-10.93). In silico analysis predicted that the substitution from W to L caused by the rs17350674 affects protein function (P< 0.05). The KIF24 rs17350674 polymorphism likely acts as a risk factor for sporadic FTLD, but a replication study would be needed to confirm these preliminary findings. © 2010 Elsevier Ireland Ltd.

Published
2010

50. DCUN1D1 is a risk factor for frontotemporal lobar degeneration

Author

Villa, C, Venturelli, E, Fenoglio, C, Clerici, F, Marcone, A, Benussi, L, Gallone, S, Scalabrini, D, Cortini, F, Serpente, M, Martinelli Boneschi, F, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti, D, Villa C., Venturelli E., Fenoglio C., Clerici F., Marcone A., Benussi L., Gallone S., Scalabrini D., Cortini F., Serpente M., Martinelli Boneschi F., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., Scarpini E., Galimberti D., Villa, C, Venturelli, E, Fenoglio, C, Clerici, F, Marcone, A, Benussi, L, Gallone, S, Scalabrini, D, Cortini, F, Serpente, M, Martinelli Boneschi, F, Cappa, S, Binetti, G, Mariani, C, Rainero, I, Giordana, M, Bresolin, N, Scarpini, E, Galimberti, D, Villa C., Venturelli E., Fenoglio C., Clerici F., Marcone A., Benussi L., Gallone S., Scalabrini D., Cortini F., Serpente M., Martinelli Boneschi F., Cappa S., Binetti G., Mariani C., Rainero I., Giordana M. T., Bresolin N., Scarpini E., and Galimberti D.

Abstract

Background and purpose: Frontotemporal lobar degeneration (FTLD) is considered as a proteinopathy; therefore, it is conceivable that genes encoding for factors involved in protein misfolding and/or degradation could play a role in its pathogenesis. Methods: An association study of defective in cullin neddylation 1 (DCN-1)-domain containing 1 (DCUN1D1), which is involved in protein degradation, was carried out in a population of 220 patients with FTLD as compared with 229 age-matched controls. Results: A statistically significant increased frequency of the GG genotype of the DCUN1D1 rs4859146 single nucleotide polymorphism (SNP) was observed in patients compared with controls (6.9 vs. 1.7%, P = 0.011, adjusted OR: 4.39, 95% CI: 1.40-13.78). Stratifying according to the clinical syndrome, significant differences were observed between the behavioral variant of frontotemporal dementia and controls (GG frequency: 6.3 vs. 1.7%, P = 0.02, OR:4.0, 95%, CI = 1.24-12.92), as well as between patients with progressive aphasia compared with controls (15.4 vs. 1.7%, P = 0.014, OR = 11.30, 95%, CI = 1.63-78.45), but not in patients with SD versus controls (8.3 vs. 1.7%, P = 0.18, OR = 5.24, 95% C.I. = 0.45-60.63). No significant differences in allelic and genotypic frequencies of the DCUN1D1 rs4859147 SNP were found. Conclusions: The GG genotype of the DCUN1D1 rs4859147 SNP represents a risk factor for the development of FTLD, increasing the risk of about fourfold. © 2009 EFNS.

Published
2009

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