JavaFX für die Energiewende: Operative Leitwarte für virtuelle Kombikraftwerke auf Basis von JavaFX

Die Energiewende ist in vollem Gange, und der Ausbau dezentraler erneuerbarer Kraftwerke gemeinsam mit intelligenter Netztechnik schreitet mit großen Schritten voran. Um bei diesem Tempo mithalten zu können und die zunehmende Komplexität zu beherrschen, bedarf es innovativer Lösungen und Tools, die die nötige Flexibilität, Skalierbarkeit und Effektivität bieten. Das Fraunhofer IWES in Kassel hat sich darauf spezialisiert, Lösungen für die Herausforderungen der heutigen und zukünftigen Energiesystemtechnik zu entwickeln und bereitzustellen. Mit JavaFX hat man ein geeignetes Mittel gefunden, dies noch ein Stück besser und schneller leisten zu können. Ein Erfahrungsbericht aus Sicht der Energiewirtschaft

Development and Application of KPIs for the Evaluation of the Control Reserve Supply by a Cross-border Renewable Virtual Power Plant .

International audienceIn an increasingly decentralised energy system with a rising share of fluctuating renewable energy sources, such as wind energy and photovoltaics (PV), the importance of virtual power plants (VPP) for the provision of ancillary services is growing. Transmission system operators (TSOs) impose stringent requirements on the reliability and accurate controllability of power plants that are susceptible to qualify for control reserve provision. It needs to be discussed whether these requirements have to evolve to enable the participation of renewables in control reserve markets. As part of the REstable research project, an innovative ICT infrastructure was set up for linking German and French wind and PV farms in a transnational VPP. Key performance indicators (KPIs) derived from technical requirements of the German TSOs are proposed and applied to quantify the quality of control reserve provision by the VPP in physical field test

Short-term Forecast of Automatic Frequency Restoration Reserve from a Renewable Energy Based Virtual Power Plant

International audienceThis paper presents the initial findings on a new forecast approach for ancillary services delivered by aggregated renewable power plants. The increasing penetration of distributed variable generators challenges grid reliability. Wind and photovoltaic power plants are technically able to provide ancillary services, but their stochastic behavior currently impedes their integration into reserve mechanisms. A methodology is developed to forecast the flexibility that a wind-photovoltaic aggregate can provide. A bivariate Kernel Density Estimator forecasts the probability to provide reserve. The methodology is tested on a case study where volumes of automatic Frequency Restoration Reserve (aFRR) are forecasted on a day-ahead horizon. It is found that the wind-photovoltaic aggregate can dedicate a limited share of its forecast production to aFRR. The frequency of insufficient reserve capacity is assessed, by comparing the capacities offered with the measured production

REstable - Improvement of renewables-based system services through better interaction of European control zones. Final report

Das Gesamtprojekt befasste sich mit zwei wichtigen Problemen, die bei Stromnetzen mit einem hohen Anteil RES (Renewable Energy Sources) auftreten können: der Bereitstellung von Systemdienstleistungen durch RES und im Kontext dessen der Erprobung von europaweiter Regelleistungsbereitstellung. Dies geschah durch die Demonstration fortschrittlicher Lösungen für ein virtuelles Kraftwerk. Der Nutzen dieser neuen Lösungen wurde außerdem unter Berücksichtigung einer verbesserten Prognose der Wetterereignisse und der kurzfristigen Vorhersage auf EU-Ebene bewertet. Hierfür werden zunächst im ersten Arbeitspaket die Szenarien, Use-Cases und KPIs erstellt, die für die Arbeiten und Analysen der weiteren Arbeitspakete benötigt werden. Im zweiten Arbeitspaket werden die Auswirkungen der Veränderung des Strommixes auf das Handelsvolumen und die Stabilität der verschiedenen Märkte anhand einer Softwaresimulation untersucht. Der letzte Teil dient schließlich dazu, die Regelleistungsbereitstellung durch ein VPP (Virtual Power Plant) in realen, internationalen Feldversuchen zu demonstrieren, zu untersuchen und zu evaluieren. Die geleisteten Vorarbeiten aus den anderen Arbeitspaketen ermöglichen an dieser Stelle das Zusammenspiel der verschiedenen Systemkomponenten und der nötigen Infrastruktur um für einen reibungslosen Ablauf der Tests zu sorgen. Innerhalb des deutschen Anteils des Gesamtprojekts wird zunächst eine bereits vorhandene Methodik zur Berechnung des Regelleistungsbedarfs an den europäischen Kontext angepasst und eine Untersuchung der FRR-Dimensionierung (Frequency Restoration Reserve) vorgenommen. Im Arbeitspaket 2 beschäftigen sich die deutschen Projektpartner weiterhin mit den Marktbarrieren und der Weiterentwicklung des FRR-Marktes im Kontext neuer Akteure mit fluktuierenden Erzeugungseinheiten. Im dritten Arbeitspaket werden schließlich die Voraussetzungen für den VPP-Betrieb und die Regelleistungsbereitstellung realisiert, als auch die physikalischen Feldtests durchgeführt und evaluiert. Hierfür müssen die Use-Cases und KPIs (Key Performance Indicators) entwickelt und definiert werden, sowie die verschiedenen Anlagen angebunden und die notwendige Software implementiert werden

Field Tests for the Provision of Frequency Containment and Frequency Restoration Reserve by Variable Renewable Energy Sources Virtual Power Plants

This paper presents the results of a series of tests on the provision of ancillary services by a Virtual Power Plant composed of variable Renewable Energy Resources. The ancillary services provided are Frequency Containment Reserve and Frequency Restoration Reserve, roughly primary and secondary reserve, and the resources used by the virtual power plant were wind and photovoltaics plant for a total of 273 MW of installed power on 17 generators. Renewable dispatchable units such as batteries, hydro or biomass were not considered in order to quantify the performance with the most critical generation technologies. Symmetric reserve activation of up to 10 MW. The quality of the reserve delivery was measured according to purpose-developed metrics and telecommunication failures were considered the main source of errors

Genomic autopsy to identify underlying causes of pregnancy loss and perinatal death

Pregnancy loss and perinatal death are devastating events for families. We assessed ‘genomic autopsy’ as an adjunct to standard autopsy for 200 families who had experienced fetal or newborn death, providing a defnitive or candidate genetic diagnosis in 105 families. Our cohort provides evidence of severe atypical in utero presentations of known genetic disorders and identifes novel phenotypes and disease genes. Inheritance of 42% of defnitive diagnoses were either autosomal recessive (30.8%), X-linked recessive (3.8%) or autosomal dominant (excluding de novos, 7.7%), with risk of recurrence in future pregnancies. We report that at least ten families (5%) used their diagnosis for preimplantation (5) or prenatal diagnosis (5) of 12 pregnancies. We emphasize the clinical importance of genomic investigations of pregnancy loss and perinatal death, with short turnaround times for diagnostic reporting and followed by systematic research follow-up investigations. This approach has the potential to enable accurate counseling for future pregnancies

Pathogenic variants in E3 ubiquitin ligase RLIM/RNF12 lead to a syndromic X-linked intellectual disability and behavior disorder

RLIM, also known as RNF12, is an X-linked E3 ubiquitin ligase acting as a negative regulator of LIM-domain containing transcription factors and participates in X-chromosome inactivation (XCI) in mice. We report the genetic and clinical findings of 84 individuals from nine unrelated families, eight of whom who have pathogenic variants in RLIM (RING finger LIM domain-interacting protein). A total of 40 affected males have X-linked intellectual disability (XLID) and variable behavioral anomalies with or without congenital malformations. In contrast, 44 heterozygous female carriers have normal cognition and behavior, but eight showed mild physical features. All RLIM variants identified are missense changes co-segregating with the phenotype and predicted to affect protein function. Eight of the nine altered amino acids are conserved and lie either within a domain essential for binding interacting proteins or in the C-terminal RING finger catalytic domain. In vitro experiments revealed that these amino acid changes in the RLIM RING finger impaired RLIM ubiquitin ligase activity. In vivo experiments in rlim mutant zebrafish showed that wild type RLIM rescued the zebrafish rlim phenotype, whereas the patient-specific missense RLIM variants failed to rescue the phenotype and thus represent likely severe loss-of-function mutations. In summary, we identified a spectrum of RLIM missense variants causing syndromic XLID and affecting the ubiquitin ligase activity of RLIM, suggesting that enzymatic activity of RLIM is required for normal development, cognition and behavior.status: publishe

The clinical and molecular spectrum of the KDM6B-related neurodevelopmental disorder

International audienceDe novo variants are a leading cause of neurodevelopmental disorders (NDDs), but because every monogenic NDD is different and usually extremely rare, it remains a major challenge to understand the complete phenotype and genotype spectrum of any morbid gene. According to OMIM, heterozygous variants in KDM6B cause "neurodevelopmental disorder with coarse facies and mild distal skeletal abnormalities." Here, by examining the molecular and clinical spectrum of 85 reported individuals with mostly de novo (likely) pathogenic KDM6B variants, we demonstrate that this description is inaccurate and potentially misleading. Cognitive deficits are seen consistently in all individuals, but the overall phenotype is highly variable. Notably, coarse facies and distal skeletal anomalies, as defined by OMIM, are rare in this expanded cohort while other features are unexpectedly common (e.g., hypotonia, psychosis, etc.). Using 3D protein structure analysis and an innovative dual Drosophila gain-of-function assay, we demonstrated a disruptive effect of 11 missense/in-frame indels located in or near the enzymatic JmJC or Zn-containing domain of KDM6B. Consistent with the role of KDM6B in human cognition, we demonstrated a role for the Drosophila KDM6B ortholog in memory and behavior. Taken together, we accurately define the broad clinical spectrum of the KDM6B-related NDD, introduce an innovative functional testing paradigm for the assessment of KDM6B variants, and demonstrate a conserved role for KDM6B in cognition and behavior. Our study demonstrates the critical importance of international collaboration, sharing of clinical data, and rigorous functional analysis of genetic variants to ensure correct disease diagnosis for rare disorders