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Characterization of the molecular dysfunctions occurring in Aicardi-Goutieres syndrome patients with mutations in ADAR1

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Genes & Diseases 2024年第3期11卷 116-119页

作者： sofan al.wardat Loredana Frasinlli Elisa Orecchini Federica Rey Silvia Anna Ciafre Silvia Galardi Jessica Garau Stella Gagliardi Simona Orcesi Davide Tonduti Stephana Carelli Cristina Cereda Ernesto Picardi alessandro Michienzi Department of Biomedicine and Prevention University of Rome Tor VergataRome 00133Italy Pediatric Clinical Research Center"Romeo ed Enrica Invernizzi" Department of Biomedical and Clinical SciencesUniversity of MilanoMilano 20157Italy Center of Functional Genomics and Rare Diseases Department of PediatricsBuzzi Children's HospitalMilano20154Italy Department of Child Neurology and Psychiatry IRccs Mondino FoundationPavia 27100Italy Unit of Pediatric Neurology C.O.A.L.A(Center for Diagnosis and Treatment of Leukodystrophies)Buzzi Children's HospitalMilano 20154Italy Department of Biosciences Biotechnologies and EnvironmentUniversityof Bari"AldoMoro"Bari 70125Italy Institute of Biomembranes Bioenergetics and Molecular Biotechnologies(IBIOM)National Research Council(CNR)Bari 70126Italy Biostructures and Biosystems National Institute(INBB) Rome00136Italy Institute of Biochemistry and Cell Biology IBBC-CNRCampus Adriano Buzzati TraversoRome 00015Italy

Aicardi-Goutieres syndrome(AGS)is a systemic inflammatory disorder caused by mutations in any one of the nine different genes,whose deficiency provokes a type I(interferon)IFN response probably central to pathogenesis.^(1) ADAR1,one of the genes mutated in AGS(AGS6),encodes for an enzyme that belongs to the ADAR family(ADAR1,ADAR2,and ADAR3)that catalyzes the conversion of adenosine to inosine within double-stranded RNAs(dsRNAs)(RNA editing A-to-1).

关键词： ADAR1 interferon res

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Assessment of VIIRS 375 m active fire using tropical peatland combustion algorithm applied to Landsat-8 over Indonesia’s peatlands

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International Journal of Digital Earth 2020年第12期13卷 1695-1716页

作者： Parwati sofan David Bruce Wilfrid Schroeder Eriita Jones Jackie Marsden Scarce Resources and Circular Economy(ScaRCE) University of South AustraliaScienceTechnologyEngineering and Mathematics(STEM)Adelaide/Mawson LakesAustralia Remote Sensing Application Center of Indonesian Institute of Aeronautics and Space(LAPAN) JakartaIndonesia National Oceanic and Atmospheric Administration(NOAA) College ParkMDUSA dImagery LandgateMidlandAustralia

VIIRS 375 m active fire data(VNP14IMG),the highest spatial resolution available cost-free fire product,were assessed for representing fire in typical degraded tropical peatlands in *** results of applying the Tropical Peatland Combustion algorithm to Landsat-8(ToPeCal-L8)daytime imagery were utilised as the fire *** permit the comparison of non-simultaneous VNP14IMG and ToPeCal-L8,peatland fire propagation speeds resulting from previous study using TET-1 data in Central Kalimantan’s peatlands were *** peatland fires were still within 750 m from their source over 15 h under uniform conditions,except for very large *** detection rates of nighttime VNP14IMG compared with ToPeCal-L8 showed about 80%agreement for small fire areas(alysis and integration of nighttime and daytime analysis of VNP14IMG data for better accuracy in fire detection for ground fire *** also demonstrate the utility of the ToPeCal-L8 algorithm with VIIRS 375 m active fire data.

关键词： Active fire assessment VIIRS 375 m active fire(VNP14IMG) ToPeCalLandsat-8 peatland fires

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