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  • 1
    Keywords: Earth sciences ; Earth Sciences ; Geophysics ; Kernkraftwerk ; Standort ; Störung ; Erdbebensicherheit ; Erdbebenbelastung ; Simulation ; Kernkraftwerk ; Bodenbewegung ; Seismizität ; Risikoanalyse ; Modellierung
    Description / Table of Contents: This volume collects several extended articles from the first workshop on Best Practices in Physics-based Fault Rupture Models for Seismic Hazard Assessment of Nuclear Installations (BestPSHANI). Held in 2015, the workshop was organized by the IAEA to disseminate the use of physics-based fault-rupture models for ground motion prediction in seismic hazard assessments (SHA). The book also presents a number of new contributions on topics ranging from the seismological aspects of earthquake cycle simulations for source scaling evaluation, seismic source characterization, source inversion and physics-based ground motion modeling to engineering applications of simulated ground motion for the analysis of seismic response of structures. Further, it includes papers describing current practices for assessing seismic hazard in terms of nuclear safety in low seismicity areas, and proposals for physics-based hazard assessment for critical structures near large earthquakes. The papers validate and verify the models by comparing synthetic results with observed data and empirical models. The book is a valuable resource for scientists, engineers, students and practitioners involved in all aspects of SHA
    Type of Medium: Online Resource
    Pages: Online-Ressource (VI, 338 p. 212 illus., 155 illus. in color, online resource)
    ISBN: 9783319727097
    Series Statement: Pageoph Topical Volumes
    Language: English
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  • 2
    Online Resource
    Online Resource
    Cham :Springer International Publishing AG,
    Keywords: Nuclear facilities. ; Electronic books.
    Type of Medium: Online Resource
    Pages: 1 online resource (333 pages)
    Edition: 1st ed.
    ISBN: 9783319727097
    Series Statement: Pageoph Topical Volumes Series
    DDC: 551.22
    Language: English
    Note: Intro -- Contents -- 1 Best Practices in Physics-Based Fault Rupture Models for Seismic Hazard Assessment of Nuclear Installations -- 1. Introduction -- 1.1. Section I: Quasi-dynamic Rupture Modeling -- 1.2. Section II: Full-Dynamic Rupture Modeling -- 1.3. Section III: Kinematic Rupture Modeling -- 1.4. Section IV: Seismic Hazard and Engineering Application -- Acknowledgements -- REFERENCES -- 2 Surface Rupture Effects on Earthquake Moment-Area Scaling Relations -- 1. Introduction -- 2. A Rate-and-State Earthquake Cycle Model -- 3. Dislocation Models -- 4. Crack Models -- 5. Conclusion -- Acknowledgements -- REFERENCES -- 3 Dynamic Rupture Modelling of the 1999 Du¨zce, Turkey Earthquake -- 1. Introduction -- 2. Model Parameters -- 2.1. Fault and Medium Model -- 2.2. Stress and Frictional Parameters -- 3. Estimation of Dynamic Parameters -- 4. Near-Source Ground Motion and Rupture Speed -- 5. Conclusion -- Acknowledgements -- REFERENCES -- 4 Dynamic Rupture Simulations Based on the Characterized Source Model of the 2011 Tohoku Earthquake -- 1. Introduction -- 2. Model -- 2.1. Characterized Model of the Tohoku Earthquake -- 2.2. Simulation Conditions -- 3. Results -- 4. Discussion -- 5. Conclusion -- Acknowledgements -- REFERENCES -- 5 Quantification of Fault-Zone Plasticity Effects with Spontaneous Rupture Simulations -- 1. Introduction -- 2. Method -- 2.1. Initial Stress and Rupture Models -- 2.2. Models of Rock-Mass Strength -- 2.3. Definition of Fault Damage Zone -- 3. Ground Motions from Layered Structure Models -- 3.1. Reduction of Average Near-Fault Ground Motions -- 3.2. Reduction of Ground Motion Extremes -- 4. Effect of Low-Velocity Zone (LVZ) on Ground Motions -- 4.1. Reduction of Near-Surface Slip and Slip Rates -- 4.2. Reduction of Near-Fault Ground Motions -- 4.3. Effect of Non-linearity and LVZ On Fault-Zone Width. , 5. Southern San Andreas Simulations -- 5.1. Results w/o LVZ -- 5.2. Results w/ LVZ -- 5.3. Comparison of Spectral Accelerations Against Ground Motion Prediction Equations -- 6. Conclusions -- Acknowledgements -- REFERENCES -- 6 Synthetic Source Inversion Tests with the Full Complexity of Earthquake Source Processes, Including Both Supershear Rupture and Slip Reactivation -- 1. Introduction -- 2. Finite Source Modeling -- 2.1. Supershear Rupture -- 2.2. Slip Reactivation -- 3. Finite Source Inversion -- 3.1. Nonlinear vs. Linear Source Inversions -- 3.2. Bayesian Source Inversion -- 3.3. Model Setups -- 4. Results -- 4.1. Supershear Rupture -- 4.2. Slip Reactivation -- 4.3. Spectral Stability Analysis -- 5. Discussion -- 6. Conclusions -- Acknowledgements -- REFERENCES -- 7 Accounting for Fault Roughness in Pseudo-Dynamic Ground-Motion Simulations -- 1. Introduction -- 2. Methods and Parameterizations -- 3. Rough-Fault Dynamic Rupture Properties -- 3.1. Rupture Dynamics for Different Rough-Fault Parameterizations -- 3.2. Parameterizing the Dynamic Source-Time Function -- 4. Ground Motions for Simplified Kinematic Rupture Models -- 4.1. Ground-Motion Characteristics for Simplified Geometrical Representations -- 4.2. Kinematic Ground-Motion Characteristics for Variations in Source-Time Function -- 5. Correlation of Kinematic Source Parameters -- 6. Discussion -- 7. Conclusions -- Acknowledgements -- REFERENCES -- 8 Within-Event and Between-Events Ground Motion Variability from Earthquake Rupture Scenarios -- 1. Introduction -- 2. UCSB Method -- 2.1. Green's Functions -- 2.2. Earthquake Source -- 2.3. Slip-Rate Function -- 2.4. 1-Point Statistics -- 2.5. 2-Point Statistics -- 2.6. Correlations Between Source Parameters -- 2.7. Ground Motion Calculation -- 3. Earthquake Rupture and Ground Motion Scenarios. , 3.1. Validation of Ground Motion Against the 2014 NGA West-2 GMPE's -- 3.2. Calculation of Ground Motion Variability -- 4. Ground Motion Variability -- 5. Conclusions -- Acknowledgements -- REFERENCES -- 9 Azimuthal Dependence of the Ground Motion Variability from Scenario Modeling of the 2014 Mw6.0 South Napa, California, Earthquake Using an Advanced Kinematic Source Model -- 1. Introduction -- 2. RIK Source Model Description -- 3. Source and Velocity Model -- 4. Results -- 4.1. Ground Motion Simulation for the South Napa Earthquake Model -- 4.2. Scenario Modeling -- 4.3. Factorization of the Ground Motion Variability -- 5. Discussion and Conclusions -- Acknowledgements -- REFERENCES -- 10 Near-Fault Broadband Ground Motion Simulations Using Empirical Green's Functions: Application to the Upper Rhine Graben (France-Germany) Case Study -- 1. Introduction -- 2. Simulations Method -- 2.1. Simulations Method: EGF Technique -- 2.2. Simulations Method: The Multi-EGF Approach -- 2.3. Simulations Method: Source Model -- 3. Upper Rhine Graben Case Study -- 4. EGF Selection and Analysis -- 5. Ground Motion Simulations -- 5.1. Sensitivity Analysis -- 5.2. Fault Geometry and Stress Drop -- 5.3. Blind Simulations -- 6. Discussion and Conclusions -- Acknowledgements -- REFERENCES -- 11 Broadband Ground Motion Simulation of the 2004 and 1977 Vrancea, Romania, Earthquakes Using Empirical Green's Function Method -- 1. Introduction -- 2. Methodology -- 2.1. Source Spectral Ratio Method and Estimation of Scaling Parameters -- 3. Data -- 4. Estimation of Source Parameters for the October 27 2004 (Mw 5.8) Earthquake -- 4.1. Source Scaling -- 5. Estimation of Source Parameters for the 1977 Mw 7.4 Earthquake -- 6. Discussion and Conclusions -- Acknowledgements -- REFERENCES -- 12 Mechanisms for Generation of Near-Fault Ground Motion Pulses for Dip-Slip Faulting. , 1. Introduction -- 1.1. Near-Fault Ground Motion Pulses -- 2. Methodology -- 2.1. Extraction of Long-Period Pulses from the Observed Ground Motions -- 2.2. Forward Simulation of Ground Motion Pulses for Normal-Faulting Event -- 3. Results -- 3.1. Effect of Ground Motion Pulses on the Response Spectra -- 3.2. Conditions for the Generation of Near-FaultGround Motion Pulses: The 2009 L'Aquila Earthquake -- 4. Discussion and Conclusions -- Acknowledgements -- REFERENCES -- 13 Performance of Irikura Recipe Rupture Model Generator in Earthquake Ground Motion Simulations with Graves and Pitarka Hybrid Approach -- 1. Introduction -- 2. Methods: IM2011 and GP2016 Rupture Model Generators -- 3. Ground Motion Simulations Using IM2011 and GP2016 Rupture Model Generators -- 3.1. Rupture Models -- 3.2. Broadband Ground Motion Method -- 3.3. Simulation Results -- 3.4. Modified IM Approach (IM-fastRS) -- 4. Conclusions -- REFERENCES -- 14 High Attenuation Rate for Shallow, Small Earthquakes in Japan -- 1. Introduction -- 2. Attenuation Characteristics of Small Earthquakes -- 2.1. Data -- 2.2. Comparison of Observations with Predictions -- 3. Attenuation of Synthetic Motion for Small and Moderate Earthquakes -- 3.1. Simulation Method and Model -- 3.2. Simulation results -- 4. Conclusions -- Acknowledgements -- REFERENCES -- 15 Stochastic Earthquake Rupture Modeling Using Nonparametric Co-Regionalization -- 1. Introduction -- 2. Correlation Between Earthquake Source Parameters -- 3. Nonparametric Co-Regionalization -- 3.1. Nonparametric Co-Regionalization -- 3.2. Modeling Examples -- 4. Stochastic Rupture Modeling -- 5. Discussion -- 6. Conclusions -- Acknowledgments -- REFERENCES -- 16 Assessment of Simulated Ground Motions in Earthquake Engineering Practice: A Case Study for Duzce (Turkey) -- 1. Introduction -- 2. Description of Frame Models. , 3. Simulation of the 12 November 1999 Duzce Earthquake (Mw = 7.1) -- 3.1. Observed Ground Motion Records -- 3.2. Simulated Ground Motion Records Obtained with Stochastic Finite-Fault Method -- 3.3. Comparison of the Simulated and Observed Ground Motion Records -- Acknowledgements -- REFERENCES -- 17 Challenges Ahead for Nuclear Facility Site-Specific Seismic Hazard Assessment in France: The Alternative Energies and the Atomic Energy Commission (CEA) Vision -- 1. Introduction -- 2. Current Practices in France for Assessing Seismic Hazard in a Nuclear Safety Approach -- 2.1. Introduction -- 2.2. The RFS 2001-01 Method -- 2.2.1 Main Steps in the RFS 2001-01 Approach -- 2.2.2 Sensitivity of the RFS 2001-01 Method to Key Steps and Parameters, and the Associated Uncertainties -- 2.3. Consequences of the Fukushima Nuclear Accident on French Nuclear Facilities: Complementary Safety Assessmentsand Requirements for Probabilistic Seismic Hazard Assessment -- 3. Key Scientific Questions and Issues -- 3.1. Seismic Source Characterization -- 3.1.1 Seismic Zoning -- 3.1.2 Fault Identification -- 3.1.3 Zoneless Approaches -- 3.1.4 Seismic Source Characterization: State of Knowledge of the Middle Durance Fault Area -- 3.2. Strong Ground Motion Prediction -- 3.2.1 Strong Motions Available in France -- 3.2.2 The RFS 2001-01 Approach: A Point Source Description and a Single Ground Motion Prediction Equation to Predict the Motion -- 3.2.3 Epistemic Uncertainty on Strong Ground Motion Prediction Accounted for in Probabilistic Seismic Hazard Assessment During Complementary Safety Assessments -- 3.2.4 Point Source Approximation Compared with Extended Fault Source Model Testing -- 3.3. On Maximum Magnitude Estimation in the Context of Slow Tectonic Activity: Methodology Proposed by the Atomic Energy Commission -- 3.3.1 Why is Mmax So Difficult to Determine?. , 3.3.2 Paleoseismological Indices, and Associated Magnitude Estimates and Uncertainties.
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  • 3
    Publication Date: 2022-02-24
    Description: In the last 10 yr, the International Atomic Energy Agency (IAEA) revised its safety standards for site evaluations of nuclear installations in response to emerging fault displacement hazard evaluation practices developed in Member States. New amendments in the revised safety guidance (DS507) explicitly recommend fault displacement hazard assessment, including separate approaches for candidate new sites versus existing sites. If there is insufficient basis to conclusively determine that a fault is not capable of surface displace- ment at an existing site, then a probabilistic fault displacement hazard analysis (PFDHA) is recommended to better characterize the hazard. This new recommendation has generated the need for the IAEA to provide its Member States with guidance on performing PFDHA, including its formulation and implementation. This article provides an overview of current PFDHA state-of-practice for nuclear installations that is consistent with the new IAEA safety standards. We also summarize progress in an ongoing international PFDHA bench- mark project that will ultimately provide technical guidance to Member States for con- ducting site-specific fault displacement hazard assessments.
    Description: Published
    Description: 2661–2672
    Description: 6T. Studi di pericolosità sismica e da maremoto
    Description: JCR Journal
    Repository Name: Istituto Nazionale di Geofisica e Vulcanologia (INGV)
    Type: article
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  • 4
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Camurati-Engelmann disease (CED, MIM 131300) is an autosomal dominant, progressive diaphyseal dysplasia characterized by hyperosteosis and sclerosis of the diaphyses of long bones. We recently assigned the CED locus to an interval between D19S422 and D19S606 at chromosome 19q13.1–q13.3 ...
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  • 5
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] We isolated NSD1 from the 5q35 breakpoint in an individual with Sotos syndrome harboring a chromosomal translocation. We identified 1 nonsense, 3 frameshift and 20 submicroscopic deletion mutations of NSD1 among 42 individuals with sporadic cases of Sotos syndrome. The results indicate that ...
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Progression through the cell cycle is catalysed by cyclin-dependent kinases (CDKs) and is negatively controlled by CDK inhibitors (CDKIs); p57KIP2 is related to p21CIP1 and p27KIP2, and is a potent tight-binding inhibitor of several G1 cyclin/CDK complexes1,2. Overexpression of p57KIP2 arrests ...
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract This paper describes a female infant with microphthalmia with linear skin defects syndrome (MLS) and monosomy for the Xp22 region. Her clinical features included right microphthalmia and sclerocornea, left corneal opacity, linear red rash and scar-like skin lesion on the nose and cheeks, and absence of the corpus callosum. Cytogenetic studies revealed a 45,X[18]/46,X,r(X)(p22q21) [24]/46,X,del(X)(p22)[58] karyotype. Fluorescence in situ hybridization analysis showed that the ring X chromosome was positive for DXZ1 and XIST and negative for the Xp and Xq telomeric regions, whereas the deleted X chromosome was positive for DXZ1, XIST, and the Xq telomeric region and negative for the Xp telomeric region. Microsatellite analysis for 19 loci at the X-differential region of Xp22 disclosed monosomy for Xp22 involving the critical region for the MLS gene, with the breakpoint between DXS1053 and DXS418. X-inactivation analysis for the methylation status of the PGK gene indicated the presence of inactive normal X chromosomes. The Xp22 deletion of our patient is the largest in MLS patients with molecularly defined Xp22 monosomy. Nevertheless, the result of X-inactivation analysis implies that the normal X chromosomes in the 46,X,del(X)(p22) cell lineage were more or less subject to X-inactivation, because normal X chromosomes in the 45,X and 46,X,r(X)(p22q21) cell lineages are unlikely to undergo X-inactivation. This supports the notion that functional absence of the MLS gene caused by inactivation of the normal X chromosome plays a pivotal role in the development of MLS in patients with Xp22 monosomy.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED) are common skeletal dysplasias with impaired enchondral ossification and premature degenerative joint disease. The two disorders were in the past considered to be distinct clinical entities; however, recent studies have proven that both diseases can result from mutations of the gene encoding cartilage oligomeric matrix protein (COMP). To characterize further COMP mutations and investigate phenotype-genotype relationships, we screened this gene in 15 patients with PSACH or MED by directly sequencing polymerase chain reaction products from genomic DNA. We identified ten mutations involving conserved residues among the eight calmodulin-like repeats of the gene product: seven were novel missense mutations in exons 9, 10, 11, 13 or 14, and the other three resulted from deletion of one of the five GAC repeats in exon 13. We have found that the GAC repeats in the 7th calmodulin-like repeat in exon 13 represent a hot-spot for mutation, and that mutations in the 7th calmodulin-like repeat produce severe PSACH phenotypes while mutations elsewhere in the gene exhibit mild PSACH or MED phenotypes. These genotype-phenotype correlations may facilitate molecular diagnosis and classification of PSACH and MED, and provide insight into the relationship between structure and function of the COMP gene product.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The pattern of X-chromosome inactivation in females is currently evaluated by assays of differential methylation in the genes between the active and the inactive X chromosomes, with methylation-sensitive enzymes. We report a new assay in the human androgen receptor (HUMARA) locus involving a methylation-specific polymerase chain reaction (M-PCR) technique, independent of the use of restriction enzymes. The assay involves the chemical modification of DNA with sodium bisulfite and subsequent PCR. By using the assay with specific primers for the methylated allele, we obtained an X-inactivation pattern based on the ratio of the maternal inactive X to the paternal inactive X. These patterns were consistent with those obtained by conventional PCR assay at the same locus in 48 female cases. We also obtained another X-inactivation pattern based on the ratio of the maternal active X to the paternal active X by using specific primers for the unmethylated allele. The latter pattern was complementary to the former pattern, and a combination of these patterns produced a reliable X-inactivation pattern. The assay revealed that 12 (11%) of the 105 normal females had non-random inactivation patterns (〉80:20 or 〈20:80). Four patients with an X; autosome translocation showed extremely non-random patterns, and these results were consistent with those obtained by previous molecular/cytogenetic studies. We conclude that M-PCR provides an accurate assay for X-inactivation and that it can be performed on various DNA samples unsuitable for restriction digestion.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Beckwith-Wiedemann syndrome (BWS) is characterized by numerous growth abnormalities and an increased risk of childhood tumors. The gene for BWS is localized in the 11p15.5 region, as determined by linkage analysis of autosomal dominant pedigrees. The increased maternal transmission pattern seen in the autosomal dominant-type pedigrees and the findings of paternal uniparental disomy reported for a subgroup of patients indicate that the gene for BWS is imprinted. Previously, we found p57 KIP2 , which is a Cdk-kinase inhibitor located at 11p15, is mutated in two BWS patients. Here, we screened for the mutation of the gene in 15 BWS patients.
    Type of Medium: Electronic Resource
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