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Aufsatz(elektronisch)#221. Juli 2020
Protecting From Malware Obfuscation Attacks Through Adversarial Risk Analysis
In: Risk analysis: an international journal, Band 40, Heft 12, S. 2598-2609
ISSN: 1539-6924
AbstractMalware constitutes a major global risk affecting millions of users each year. Standard algorithms in detection systems perform insufficiently when dealing with malware passed through obfuscation tools. We illustrate this studying in detail an open source metamorphic software, making use of a hybrid framework to obtain the relevant features from binaries. We then provide an improved alternative solution based on adversarial risk analysis which we illustrate describe with an example.
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Open Access#32021
Structured Expert Judgement Issues in a Supply Chain Cyber Risk Management System
The authors acknowledge the support by the Spanish Ministry of Economy and Innovation program MTM2017-86875-C3-1-R and RTC-2017-6593-7, the ESF-COST Action IS1304 on Expert Judgement and the AXA-ICMAT Chair on Adversarial Risk Analysis. This work has also been partially supported by the Spanish Ministry of Economy and Competitiveness, through the "Severo Ochoa" Program for Centers of Excellence in R&D (SEV-2015-0554), and has received funding from the European Union's H2020 Program for Research, Technological Development and Demonstration, under grant agreement no. 740920 (CYBECO). AR work acknowledges the Spanish Ministry of Economy for his PTA position. FR acknowledges the support of the Chair of Excellence of the Comunidad de Madrid. FR and DRI acknowledge the support of the US National Science Foundation through grant DMS-163851.
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Open Access#42021
Association of Variants in the SPTLC1 Gene with Juvenile Amyotrophic Lateral Sclerosis
In: Johnson , J O , Chia , R , Miller , D E , Li , R , Kumaran , R , Abramzon , Y , Alahmady , N , Renton , A E , Topp , S D , Gibbs , J R , Cookson , M R , Sabir , M S , Dalgard , C L , Troakes , C , Jones , A R , Shatunov , A , Iacoangeli , A , Al Khleifat , A , Ticozzi , N , Silani , V , Gellera , C , Blair , I P , Dobson-Stone , C , Kwok , J B , Bonkowski , E S , Palvadeau , R , Tienari , P J , Morrison , K E , Shaw , P J , Al-Chalabi , A , Brown , R H , Calvo , A , Mora , G , Al-Saif , H , Gotkine , M , Leigh , F , Chang , I J , Perlman , S J , Glass , I , Scott , A I , Shaw , C E , Basak , A N , Landers , J E , Chiò , A , Crawford , T O , Smith , B N , Traynor , B J , Smith , B N , Ticozzi , N , Fallini , C , Gkazi , A S , Topp , S D , Scotter , E L , Kenna , K P , Keagle , P , Tiloca , C , Vance , C , Troakes , C , Colombrita , C , King , A , Pensato , V , Castellotti , B , Baas , F , Ten Asbroek , A L M A , McKenna-Yasek , D , McLaughlin , R L , Polak , M , Asress , S , Esteban-Pérez , J , Stevic , Z , D'Alfonso , S , Mazzini , L , Comi , G P , Del Bo , R , Ceroni , M , Gagliardi , S , Querin , G , Bertolin , C , Van Rheenen , W , Rademakers , R , Van Blitterswijk , M , Lauria , G , Duga , S , Corti , S , Cereda , C , Corrado , L , Sorarù , G , Williams , K L , Nicholson , G A , Blair , I P , Leblond-Manry , C , Rouleau , G A , Hardiman , O , Morrison , K E , Veldink , J H , Van Den Berg , L H , Al-Chalabi , A , Pall , H , Shaw , P J , Turner , M R , Talbot , K , Taroni , F , García-Redondo , A , Wu , Z , Glass , J D , Gellera , C , Ratti , A , Brown , R H , Silani , V , Shaw , C E , Landers , J E , Dalgard , C L , Adeleye , A , Soltis , A R , Alba , C , Viollet , C , Bacikova , D , Hupalo , D N , Sukumar , G , Pollard , H B , Wilkerson , M D , Martinez , E M G , Abramzon , Y , Ahmed , S , Arepalli , S , Baloh , R H , Bowser , R , Brady , C B , Brice , A , Broach , J , Campbell , R H , Camu , W , Chia , R , Cooper-Knock , J , Ding , J , Drepper , C , Drory , V E , Dunckley , T L , Eicher , J D , England , B K , Faghri , F , Feldman , E , Floeter , M K , Fratta , P , Geiger , J T , Gerhard , G , Gibbs , J R , Gibson , S B , Glass , J D , Hardy , J , Harms , M B , Heiman-Patterson , T D , Hernandez , D G , Jansson , L , Kirby , J , Kowall , N W , Laaksovirta , H , Landeck , N , Landi , F , Le Ber , I , Lumbroso , S , Macgowan , D J L , Maragakis , N J , Mora , G , Mouzat , K , Murphy , N A , Myllykangas , L , Nalls , M A , Orrell , R W , Ostrow , L W , Pamphlett , R , Pickering-Brown , S , Pioro , E P , Pletnikova , O , Pliner , H A , Pulst , S M , Ravits , J M , Renton , A E , Rivera , A , Robberecht , W , Rogaeva , E , Rollinson , S , Rothstein , J D , Scholz , S W , Sendtner , M , Shaw , P J , Sidle , K C , Simmons , Z , Singleton , A B , Smith , N , Stone , D J , Tienari , P J , Troncoso , J C , Valori , M , Van Damme , P , Van Deerlin , V M , Van Den Bosch , L , Zinman , L , Landers , J E , Chiò , A , Traynor , B J , Angelocola , S M , Ausiello , F P , Barberis , M , Bartolomei , I , Battistini , S , Bersano , E , Bisogni , G , Borghero , G , Brunetti , M , Cabona , C , Calvo , A , Canale , F , Canosa , A , Cantisani , T A , Capasso , M , Caponnetto , C , Cardinali , P , Carrera , P , Casale , F , Chiò , A , Colletti , T , Conforti , F L , Conte , A , Conti , E , Corbo , M , Cuccu , S , Dalla Bella , E , D'Errico , E , Demarco , G , Dubbioso , R , Ferrarese , C , Ferraro , P M , Filippi , M , Fini , N , Floris , G , Fuda , G , Gallone , S , Gianferrari , G , Giannini , F , Grassano , M , Greco , L , Iazzolino , B , Introna , A , La Bella , V , Lattante , S , Lauria , G , Liguori , R , Logroscino , G , Logullo , F O , Lunetta , C , Mandich , P , Mandrioli , J , Manera , U , Manganelli , F , Marangi , G , Marinou , K , Marrosu , M G , Martinelli , I , Messina , S , Moglia , C , Mora , G , Mosca , L , Murru , M R , Origone , P , Passaniti , C , Petrelli , C , Petrucci , A , Pozzi , S , Pugliatti , M , Quattrini , A , Ricci , C , Riolo , G , Riva , N , Russo , M , Sabatelli , M , Salamone , P , Salivetto , M , Salvi , F , Santarelli , M , Sbaiz , L , Sideri , R , Simone , I , Simonini , C , Spataro , R , Tanel , R , Tedeschi , G , Ticca , A , Torriello , A , Tranquilli , S , Tremolizzo , L , Trojsi , F , Vasta , R , Vacchiano , V , Vita , G , Volanti , P , Zollino , M & Zucchi , E 2021 , ' Association of Variants in the SPTLC1 Gene with Juvenile Amyotrophic Lateral Sclerosis ' , JAMA neurology . https://doi.org/10.1001/jamaneurol.2021.2598
Johnson, Janel O; Chia, Ruth; Miller, Danny E; Li, Rachel; Kumaran, Ravindran; Abramzon, Yevgeniya; Alahmady, Nada; Renton, Alan E
Johnson, Janel O; Chia, Ruth; Miller, Danny E; Li, Rachel; Kumaran, Ravindran; Abramzon, Yevgeniya; Alahmady, Nada; Renton, Alan E; Topp, Simon D; Gibbs, J. Raphael; Cookson, Mark R; Sabir, Marya S; Dalgard, Clifton L; Troakes, Claire; Jones, Ashley R; Shatunov, Aleksey; Iacoangeli, Alfredo; Al Khleifat, Ahmad; Ticozzi, Nicola; Silani, Vincenzo; Gellera, Cinzia; Blair, Ian P; Dobson-Stone, Carol; Kwok, John B; Bonkowski, Emily S; Palvadeau, Robin; Tienari, Pentti J; Morrison, Karen E; Shaw, Pamela J; Al-Chalabi, Ammar; Brown, Robert H; Calvo, Andrea; Mora, Gabriele; Al-Saif, Hind; Gotkine, Marc; Leigh, Fawn; Chang, Irene J; Perlman, Seth J; Glass, Ian; Scott, Anna I; Shaw, Christopher E; Basak, A. Nazli; Landers, John E; Chiò, Adriano; Crawford, Thomas O; Smith, Bradley N; Traynor, Bryan J; Fallini, Claudia; Gkazi, Athina Soragia; Scotter, Emma L; Kenna, Kevin P; Keagle, Pamela; Tiloca, Cinzia; Vance, Caroline; Colombrita, Claudia; King, Andrew; Pensato, Viviana; Castellotti, Barbara; Baas, Frank; Ten Asbroek, Anneloor L.M.A; McKenna-Yasek, Diane; McLaughlin, Russell L; Polak, Meraida; Asress, Seneshaw; Esteban-Pérez, Jesús; Stevic, Zorica; D'Alfonso, Sandra; Mazzini, Letizia; Comi, Giacomo P; Del Bo, Roberto; Ceroni, Mauro; Gagliardi, Stella; Querin, Giorgia; Bertolin, Cinzia; Van Rheenen, Wouter; Rademakers, Rosa; Van Blitterswijk, Marka; Lauria, Giuseppe; Duga, Stefano; Corti, Stefania; Cereda, Cristina; Corrado, Lucia; Sorarù, Gianni; Williams, Kelly L; Nicholson, Garth A; Leblond-Manry, Claire; Rouleau, Guy A; Hardiman, Orla; Veldink, Jan H; Van Den Berg, Leonard H; Pall, Hardev; Turner, Martin R; Talbot, Kevin; Taroni, Franco; García-Redondo, Alberto; Wu, Zheyang; Glass, Jonathan D; Ratti, Antonia; Adeleye, Adelani; Soltis, Anthony R; Alba, Camille; Viollet, Coralie; Bacikova, Dagmar; Hupalo, Daniel N; Sukumar, Gauthaman; Pollard, Harvey B; Wilkerson, Matthew D; Martinez, Elisa Mc Grath; Ahmed, Sarah; Arepalli, Sampath; Baloh, Robert H; Bowser, Robert; Brady, Christopher B; Brice, Alexis; Broach, James; Campbell, Roy H; Camu, William; Cooper-Knock, John; Ding, Jinhui; Drepper, Carsten; Drory, Vivian E; Dunckley, Travis L; Eicher, John D; England, Bryce K; Faghri, Faraz; Feldman, Eva; Floeter, Mary Kay; Fratta, Pietro; Geiger, Joshua T; Gerhard, Glenn; Gibson, Summer B; Hardy, John; Harms, Matthew B; Heiman-Patterson, Terry D; Hernandez, Dena G; Jansson, Lilja; Kirby, Janine; Kowall, Neil W; Laaksovirta, Hannu; Landeck, Natalie; Landi, Francesco; Le Ber, Isabelle; Lumbroso, Serge; Macgowan, Daniel J.L; Maragakis, Nicholas J; Mouzat, Kevin; Murphy, Natalie A; Myllykangas, Liisa; Nalls, Mike A; Orrell, Richard W; Ostrow, Lyle W; Pamphlett, Roger; Pickering-Brown, Stuart; Pioro, Erik P; Pletnikova, Olga; Pliner, Hannah A; Pulst, Stefan M; Ravits, John M; Rivera, Alberto; Robberecht, Wim; Rogaeva, Ekaterina; Rollinson, Sara; Rothstein, Jeffrey D; Scholz, Sonja W; Sendtner, Michael; Sidle, Katie C; Simmons, Zachary; Singleton, Andrew B; Smith, Nathan; Stone, David J; Troncoso, Juan C; Valori, Miko; Van Damme, Philip; Van Deerlin, Vivianna M; Van Den Bosch, Ludo; Zinman, Lorne; Angelocola, Stefania M; Ausiello, Francesco P; Barberis, Marco; Bartolomei, Ilaria; Battistini, Stefania; Bersano, Enrica; Bisogni, Giulia; Borghero, Giuseppe; Brunetti, Maura; Cabona, Corrado; Canale, Fabrizio; Canosa, Antonio; Cantisani, Teresa A; Capasso, Margherita; Caponnetto, Claudia; Cardinali, Patrizio; Carrera, Paola; Casale, Federico; Colletti, Tiziana; Conforti, Francesca L; Conte, Amelia; Conti, Elisa; Corbo, Massimo; Cuccu, Stefania; Dalla Bella, Eleonora; D'Errico, Eustachio; Demarco, Giovanni; Dubbioso, Raffaele; Ferrarese, Carlo; Ferraro, Pilar M; Filippi, Massimo; Fini, Nicola; Floris, Gianluca; Fuda, Giuseppe; Gallone, Salvatore; Gianferrari, Giulia; Giannini, Fabio; Grassano, Maurizio; Greco, Lucia; Iazzolino, Barbara; Introna, Alessandro; La Bella, Vincenzo; Lattante, Serena; Liguori, Rocco; Logroscino, Giancarlo; Logullo, Francesco O; Lunetta, Christian; Mandich, Paola; Mandrioli, Jessica; Manera, Umberto; Manganelli, Fiore; Marangi, Giuseppe; Marinou, Kalliopi; Marrosu, Maria Giovanna; Martinelli, Ilaria; Messina, Sonia; Moglia, Cristina; Mosca, Lorena; Murru, Maria R; Origone, Paola; Passaniti, Carla; Petrelli, Cristina; Petrucci, Antonio; Pozzi, Susanna; Pugliatti, Maura; Quattrini, Angelo; Ricci, Claudia; Riolo, Giulia; Riva, Nilo; Russo, Massimo; Sabatelli, Mario; Salamone, Paolina; Salivetto, Marco; Salvi, Fabrizio; Santarelli, Marialuisa; Sbaiz, Luca; Sideri, Riccardo; Simone, Isabella; Simonini, Cecilia; Spataro, Rossella; Tanel, Raffaella; Tedeschi, Gioacchino; Ticca, Anna; Torriello, Antonella; Tranquilli, Stefania; Tremolizzo, Lucio; Trojsi, Francesca; Vasta, Rosario; Vacchiano, Veria; Vita, Giuseppe; Volanti, Paolo; Zollino, Marcella; Zucchi, Elisabetta
Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation. Objective: To identify the genetic variants associated with juvenile ALS. Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism. Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members. Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway. Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.
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