Amprion

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Anderski T.,Amprion | Careri F.,RSE SpA | Migliavacca G.,RSE SpA | Grisey N.,French Electricity Transmission Network | And 6 more authors.
2016 IEEE International Energy Conference, ENERGYCON 2016 | Year: 2016

By 2050, the European Union aims to cut the European CO2 emissions by 80-95% compared to 1990. To reach this target, the European power grid plays a key role to ensure both a low-carbon and affordable electricity supply. In this context, the e-Highway2050 project identifies the European grid architectures, which would be needed under five contrasted low-carbon energy scenarios. An innovative approach based on Monte-Carlo simulations of the European power system, modeled with approximately one hundred zones, ensures both robustness of the results and the possibility to consider all of Europe at once. A detailed cost-benefit analysis of the different grid architectures shows their high profitability. Their implementation between 2030 and 2050 in terms of transmission requirements and technological solutions is discussed. © 2016 IEEE.


News Article | December 21, 2016
Site: www.scientificamerican.com

Eating beef from an animal infected with mad cow disease can lead to an untreatable condition that attacks the brain and is universally fatal, but symptoms can take decades to emerge. Thankfully, a new blood-screening technology can spot the condition, known as variant Creutzfeldt-Jakob disease, with 100 percent accuracy, perhaps years before it attacks. Misfolded proteins called prions cause both mad cow and variant Creutzfeldt-Jakob disease. Once they invade the brain, they begin recruiting normal proteins and forcing them to adopt the same abnormal shape. The prions and the blighted proteins clump together forming increasingly large aggregate deposits that wreak havoc on the brain and invariably lead to death. The disease, however, has a long incubation period. “In the case of humans, the estimation goes from several years to a few decades,” says Claudio Soto, a neurologist at McGovern Medical School at UTHealth in Houston. “So it could be that you're exposed one day and then, 40 years after, you develop the disease.” In the interim, the prions hang out in non-brain tissues such as the appendix and tonsils, and because they do not cause symptoms, the infected person becomes a silent carrier. Perhaps the worst outbreak of variant Creutzfeldt-Jakob disease occurred in the United Kingdom during the 1980s and 1990s, when large swaths of the population were exposed to beef contaminated with mad cow disease. Since then, there have been 277 cases in the U.K. and an epidemiological study published in 2013 estimates that another 1 in 2,000 people there, about 30,000 in total, are silent carriers. While it is not clear how many of these people will go on to develop the disease, blood donations from silent carriers could jeopardize the country’s blood supply, according to Soto. The new screening test stands to alleviate the uncertainty, however. Soto led the team behind one of two studies published today in Science Translational Medicine that assessed a diagnostic test for variant Creutzfeldt-Jakob disease, which can detect very low levels of prion proteins in the blood. Soto’s team and a team led by Daisy Bougard of the French Blood Establishment in Montpellier, France ran the test on blood samples from variant Creutzfeldt-Jakob disease patients in the U.K. and France. The two teams used slightly different methods, but the basic idea was the same: the test essentially mimics the progression of the disease in an accelerated, artificial environment. First the prion proteins are separated from the blood and combined with normal proteins, which take on an abnormal shape, forming aggregate clumps. Then, the aggregates are pulled apart and recombined with more normal proteins. The process is repeated over and over again, in effect replicating the prion proteins until very small quantities are amplified enough to be easily detected. If there are no prions present in the blood, nothing happens. Between the two studies, the test was able to identify a total of 32 cases of variant Creutzfeldt-Jakob disease with 100% percent accuracy, and there were no false positives among the 391 controls, which included regular blood donors, patients with a different form of Creutzfeldt-Jakob disease, and patients with other neurological diseases. In addition, Bougard’s group was able to diagnose variant Creutzfeldt-Jakob disease in the blood of two patients 1.3 and 2.6 years before they developed clinical symptoms. The results not only confirm that the test can accurately diagnose the disease, but also suggest that it may be able make the diagnosis before patients develop symptoms, which could be particularly important in places like the U.K. with a large number of silent carriers. The test will “improve the safety of our blood supply [by eliminating] any blood samples that may be potentially contaminated with prions,” says Soto, “which will in turn diminish the possibility of people getting infected, and eventually developing this fatal disease.” Soto founded a startup called Amprion to develop the technology. The company is currently working on optimizing the test and obtaining approval in the United States and Europe to use it for blood screening. Soto expects it to be commercially available within one to two years. Paul Brown, formerly a senior investigator in the Laboratory of Central Nervous System Studies at the National Institutes of Health, who was not involved in the studies praised the accuracy of the test, saying that the two papers “reflect the immense progress that testing has undergone during the past decade.” However, he pointed out that variant Creutzfeldt-Jakob disease is no longer a major global problem, with only two cases in 2016, and said the test would have been more useful during the peak of the epidemic. Christina Orrú, a neurologist at the National Institutes of Health, who was not involved in the studies, thinks the test is still needed to screen and monitor prion contamination in the blood supply. “We currently don’t really understand the implications of silent carriers and if they could harbor another wave of variant Creutzfeldt-Jakob disease, particularly in the U.K.,” she says. Sylvain Lehmann, a neurologist at France’s National Center for Scientific Research, who was not involved in the studies was also impressed by the sensitivity and specificity of the test, saying that the findings confirm that silent carriers can transmit the disease. Because the current versions of the test are complicated, labor-intensive and time consuming, they are not necessarily suitable for screening large volumes of blood, he says, but they could be useful for confirming a diagnosis, especially in silent carriers, or detecting variant Creutzfeldt-Jakob disease in at-risk patients who received a contaminated blood transfusion. The test may also have other applications. Soto and Bougard point out that while variant Creutzfeldt-Jakob disease is rare, more common neurodegenerative disorders like Alzheimer’s and Parkinson’s are caused by a similar process of misfolded proteins aggregating in the brain and causing damage. Both are hopeful that the new test can be adapted to screen for these diseases at an early stage, before symptoms occur. “This is a big problem with all these brain diseases, that when the disease manifests clinically, it's always very late, and at the time that the brain is largely destroyed,” says Soto. “We are trying to develop a blood test to detect Alzheimer's disease and Parkinson's disease before the clinical symptoms of the disease appear so we have better possibilities for therapies to work.”


Holttinen H.,VTT Technical Research Center of Finland | Orths A.G.,Energinet.dk | Orths A.G.,Otto Von Guericke University of Magdeburg | Eriksen P.B.,Energinet.dk | And 8 more authors.
IEEE Power and Energy Magazine | Year: 2011

The power systems in denmark, portugal, Spain, Ireland, and Germany have some of the highest wind penetrations in the world, as shown in Table 1. © 2011 IEEE.


Herrmann H.-J.,Siemens AG | Kuhn H.,Transpower | Fohring H.,Amprion | Ludwig A.,50Hertz Transmission GmbH | And 2 more authors.
43rd International Conference on Large High Voltage Electric Systems 2010, CIGRE 2010 | Year: 2010

This paper addresses the requirements of renewable generation in terms of protection concepts and also covers technical solutions surrounding disconnection of cogeneration plants. The German practice is introduced. Three guidelines exist as general rules. These describe the required boundary conditions. The first guideline addresses the general rules for connection of generation on the transmission network. The second addresses the connection conditions for infeed in medium voltage and the third for infeed in high voltage networks. The main renewable energy resource in Germany is wind, where the installed capacity reaches approximately 25 GW. The size of wind farms comes close to that of large power plant units being some hundred megawatts. Therefore this paper focuses on new challenges with renewable generation via wind farms. Due to the large installed power base, the dynamic behavior of such systems becomes very important. The following requirements must be fulfilled: - No immediate disconnection of the generation in the case of a network fault (coordinated disconnection is necessary) - Supporting of the line voltage during the fault via supply of reactive power - After fault clearing the consumption of reactive power must be the same as before. The generation must contribute the voltage stability by delivering reactive power. - One important rule is that the generation must remain connected for at least 150 ms to the system during voltage dips (fault-ride-through). The renewable generation must contribute to the fault clearing by delivering a short circuit current. The amount depends on the generation principle. Squirrel cage induction and double fed induction generators (DFIG) can deliver a fault current equal to a multiple of the rated current while via full converter design the short circuit current is smaller (close to the rated current). In typical examples the required technical solutions of the protection and disconnection concepts will be explained. The disconnection criteria are graded under- and overvoltage functions on the different voltage levels as well as over- and underfrequency functions based on typical frequency limits (f<: 47.5 Hz; f>: 51.5 Hz).


News Article | December 7, 2016
Site: www.eurekalert.org

HOUSTON - (Dec. 6, 2016) - Misfolded proteins associated with Parkinson's disease were detected in cerebrospinal fluid by scientists at McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), paving the way to development of a biochemical test to diagnosis the disease. The research, led by Claudio Soto, Ph.D., professor in the Department of Neurology and the director of the George and Cynthia Mitchell Center for Alzheimer's disease and Related Brain Disorders at UTHealth, was published in yesterday's issue of JAMA Neurology, a journal of the American Medical Association. Parkinson's disease (PD) is a degenerative disorder of the brain that initially affects motor skills, causing tremors, stiffness, slowness of movement and impaired balance. As it progresses, patients may develop cognitive problems, psychiatric alterations and dementia. There are no current laboratory or blood tests that have been proven to help in diagnosis. Because the disease can be difficult to diagnose accurately, diagnosis is sometimes is made by ruling out other neurological diseases. Using a technology developed by Soto that was shown in previous studies to detect misfolded proteins associated with diseases such as Creutzfeld-Jacob and Alzheimer's disease, researchers targeted misfolded alpha-synuclein (aSyn) aggregates as a way of developing a sensitive biochemical diagnosis for PD. The Protein Misfolding Cyclic Amplification (PMCA) technology was able to detect very small amounts of the misfolded protein circulating in cerebrospinal fluid. "Of significant interest is that the amount of aSyn detected correlates with the severity of the disease and in two of the control samples, aSyn was detected and those people later developed clinical symptoms of PD," Soto said. The research included blind screenings of cerebrospinal fluid of two cohorts of 76 PD patients, as well as controls of 65 people who were healthy or affected by other neurological disorders, 18 affected by neurodegenerative diseases and 14 affected by Alzheimer's disease. Since cerebrospinal fluid is removed through spinal taps, which are invasive and painful, the hope is that future research would enable optimization of the PMCA assay to detect aSyn in blood or urine. "The hope is that we could use aSyn-PMCA to detect PD in patients before they develop symptoms, and those patients could be entered into clinical trials for novel treatments that might prevent, cure or delay the progression of the disease before substantial and irreversible damage of the brain," Soto said. The research was funded in part by grants from the Michael J. Fox Foundation for Parkinson's Research. The first author of the paper is Mohammad Shahnawaz, Ph.D., of McGovern Medical School at UTHealth. Co-authors are from Kyoto Prefectural University of Medicine in Kyoto, Japan; Higashi Matsudo Municipal Hospital in Matsudo, Japan; Paracelsus-Elena-Klinik Kassel in Kassel, Germany; and University Medical Center Göttingen in Göttingen, Germany. Soto is an inventor on patented PMCA technology and is currently the founder, chief scientific officer and vice president of Amprion Inc., a biotech company focusing on the commercial utilization of PMCA for diagnosis. Soto and Shahnawaz are inventors in patented technology on the use of aSyn-PMCA for PD diagnosis.


Schneiders C.,Amprion | Vanzetta J.,Amprion | Verstege J.F.,University of Wuppertal
IEEE PES Innovative Smart Grid Technologies Conference Europe | Year: 2012

One major task of the control centre operator is to identify the global system state of the transmission system. However, during the past couple of years, the complexity of transmission system operation has increased significantly. The operator in the control room has to observe wider external areas to fully evaluate system security. Along with the development of information technology and functionality of SCADA systems, the amount of data the operator has to monitor has augmented notably. The focus of this work is to develop a visualization of the global system state and to analyse and enhance situation awareness in wide area electrical transmission systems. In addition first versions of this new MMI are implemented into the SCADA system of the German TSO, Amprion. Thereby the study also enables usability engineering of variant new visualizations in the control centre. The proposed solution allows the operator to observe the global system state at a glance and enables intuitive situation awareness by improving visual perception like pattern recognition. © 2012 IEEE.


Vanzetta J.,Amprion | Schneiders C.,Amprion
IEEE PES Innovative Smart Grid Technologies Conference Europe | Year: 2011

During the past couple of years, the complexity of transmission system operation has increased significantly. Along with the liberalization of the electricity markets a broad range of new, varying tasks have arisen and with this a significant increase of interfaces which the operators in control centres have to cope with. Furthermore the substantial growth of volatile wind and PV energy sources and large-scale energy trades across wide-areas lead to frequently changing load flow situations. As a consequence of these huge load flows, the power system is more frequently operated ever closer to its security limits. This situation is further aggravated by recent developments as the so called Nuclear Power Plant (NPP) Moratorium in Germany came in effect as a reaction to the Japanese disaster in Fukushima. The following paper will provide an overview of the current challenges facing Amprion, one of the four Transmission System Operator (TSO) in Germany, and will present examples of solution approaches. © 2011 IEEE.


Patent
Board Of Regents Of The University Of Texas System and Amprion | Date: 2015-09-11

Methods and kits are provided for amplifying and detecting A proteins from samples, for example, from patients having Alzheimers Disease. For example, a method for determining a presence of a soluble, misfolded A protein may include contacting the sample with a monomeric, folded A protein to form an incubation mixture; conducting an incubation cycle two or more times on the incubation mixture effective to form an amplified portion of misfolded A protein; incubating the incubation mixture effective to cause misfolding and/or aggregation of at least a portion of the monomeric, folded A protein; physically disrupting the incubation mixture effective to at least partly de-aggregate at least a portion of a misfolded A aggregate present; and determining the presence of the soluble, misfolded A protein in the sample by detecting at least a portion of the amplified portion of misfolded A protein.


Patent
Board Of Regents Of The University Of Texas System and Amprion | Date: 2015-09-11

Methods and kits are provided for amplifying and detecting S proteins from samples, for example, from patients having Parkinsons Disease. For example, a method for determining a presence of a soluble, misfolded S protein may include: contacting the sample with a monomeric, folded S protein to form an incubation mixture; conducting an incubation cycle two or more times on the incubation mixture effective to form an amplified portion of misfolded S protein; incubating the incubation mixture effective to cause misfolding and/or aggregation of at least a portion of the monomeric, folded S protein in the presence of the soluble, misfolded S protein; physically disrupting the incubation mixture effective to at least partly de-aggregate at least a portion of a misfolded S aggregate present; and determining the presence of the soluble, misfolded S protein in the sample by detecting at least a portion of the soluble, misfolded S protein.


Patent
Board Of Regents Of The University Of Texas System and Amprion | Date: 2015-09-11

Methods and kits are provided for amplifying and detecting misfolded proteins from samples, for example, from patients having Alzheimers Disease, Parkinsons Disease, and the like. For example, a method for determining a presence of soluble, misfolded protein in a sample may include contacting the sample with a monomeric, folded protein to form an incubation mixture; conducting an incubation cycle two or more times effective to form an amplified portion of misfolded protein; incubating the incubation mixture effective to cause misfolding and/or aggregation of at least a portion of the monomeric, folded protein; physically disrupting the incubation mixture effective to break up at least a portion of any protein aggregate present; and determining the presence of the soluble, misfolded protein in the sample by detecting at least a portion of the soluble, misfolded protein. The monomeric, folded protein and the soluble, misfolded protein may exclude prion protein (PrP) and isoforms thereof.

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