Niemann–Pick disease type C1 presenting with psychosis in an adolescent male

Niemann–Pick disease, a neurovisceral lysosomal lipid storage disorder, is a rare disorder that is unknown to many clinicians. The disease, that often has its onset during childhood or adolescence, shows a polymorphic clinical picture, including psychiatric symptoms. Because of its infrequence, Niemann–Pick disease is diagnosed with an average delay of 6 years. This report presents a case of an adolescent male whose symptoms had led to various hospitalisations and psychiatric diagnoses. When he presented with psychotic symptoms in our department, thorough diagnosis revealed Niemann–Pick disease type C1 as the underlying disease

BKG/DGFI Combination Center Annual Report 2012

This report summarizes the activities of the Federal Agency for Cartography and Geodesy (Bundesamt fuer Kartographie und Geodaesie, BKG) and the German Geodetic Research Institute (Deutsches Geodaetisches Forschungsinstitut, DGFI)BKG/DGFI Combination Center in 2011 and outlines the planned activities for the year 2012. The main focus was to stabilize outlier detection and to update the Web presentation of the combined products

Absence of Erythrocyte Sequestration and Lack of Multicopy Gene Family Expression in Plasmodium falciparum from a Splenectomized Malaria Patient

BACKGROUND:To avoid spleen-dependent killing mechanisms parasite-infected erythrocytes (IE) of Plasmodium falciparum malaria patients have the capacity to bind to endothelial receptors. This binding also known as sequestration, is mediated by parasite proteins, which are targeted to the erythrocyte surface. Candidate proteins are those encoded by P. falciparum multicopy gene families, such as var, rif, stevor or PfMC-2TM. However, a direct in vivo proof of IE sequestration and expression of multicopy gene families is still lacking. Here, we report on the analysis of IE from a black African immigrant, who received the diagnosis of a malignant lymphoproliferative disorder and subsequently underwent splenectomy. Three weeks after surgery, the patient experienced clinical falciparum malaria with high parasitemia and circulating developmental parasite stages usually sequestered to the vascular endothelium such as late trophozoites, schizonts or immature gametocytes. METHODOLOGY/PRINCIPAL FINDINGS:Initially, when isolated from the patient, the infected erythrocytes were incapable to bind to various endothelial receptors in vitro. Moreover, the parasites failed to express the multicopy gene families var, A-type rif and stevor but expression of B-type rif and PfMC-2TM genes were detected. In the course of in vitro cultivation, the parasites started to express all investigated multicopy gene families and concomitantly developed the ability to adhere to endothelial receptors such as CD36 and ICAM-1, respectively. CONCLUSION/SIGNIFICANCE:This case strongly supports the hypothesis that parasite surface proteins such as PfEMP1, A-type RIFIN or STEVOR are involved in interactions of infected erythrocytes with endothelial receptors mediating sequestration of mature asexual and immature sexual stages of P. falciparum. In contrast, multicopy gene families coding for B-type RIFIN and PfMC-2TM proteins may not be involved in sequestration, as these genes were transcribed in infected but not sequestered erythrocytes

Gemfibrozil-Induced Intracellular Triglyceride Increase in SH-SY5Y, HEK and Calu-3 Cells

Gemfibrozil is a drug that has been used for over 40 years to lower triglycerides in blood. As a ligand for peroxisome proliferative-activated receptor-alpha (PPARα), which is expressed in many tissues, it induces the transcription of numerous genes for carbohydrate and lipid-metabolism. However, nothing is known about how intracellular lipid-homeostasis and, in particular, triglycerides are affected. As triglycerides are stored in lipid-droplets, which are known to be associated with many diseases, such as Alzheimer’s disease, cancer, fatty liver disease and type-2 diabetes, treatment with gemfibrozil could adversely affect these diseases. To address the question whether gemfibrozil also affects intracellular lipid-levels, SH-SY5Y, HEK and Calu-3 cells, representing three different metabolically active organs (brain, lung and kidney), were incubated with gemfibrozil and subsequently analyzed semi-quantitatively by mass-spectrometry. Importantly, all cells showed a strong increase in intracellular triglycerides (SH-SY5Y: 170.3%; HEK: 272.1%; Calu-3: 448.1%), suggesting that the decreased triglyceride-levels might be due to an enhanced cellular uptake. Besides the common intracellular triglyceride increase, a cell-line specific alteration in acylcarnitines are found, suggesting that especially in neuronal cell lines gemfibrozil increases the transport of fatty acids to mitochondria and therefore increases the turnover of fatty acids for the benefit of additional energy supply, which could be important in diseases, such as Alzheimer’s disease

Methylxanthines Induce a Change in the AD/Neurodegeneration-Linked Lipid Profile in Neuroblastoma Cells

Alzheimer’s disease (AD) is characterized by an increased plaque burden and tangle accumulation in the brain accompanied by extensive lipid alterations. Methylxanthines (MTXs) are alkaloids frequently consumed by dietary intake known to interfere with the molecular mechanisms leading to AD. Besides the fact that MTX consumption is associated with changes in triglycerides and cholesterol in serum and liver, little is known about the effect of MTXs on other lipid classes, which raises the question of whether MTX can alter lipids in a way that may be relevant in AD. Here we have analyzed naturally occurring MTXs caffeine, theobromine, theophylline, and the synthetic MTXs pentoxifylline and propentofylline also used as drugs in different neuroblastoma cell lines. Our results show that lipid alterations are not limited to triglycerides and cholesterol in the liver and serum, but also include changes in sphingomyelins, ceramides, phosphatidylcholine, and plasmalogens in neuroblastoma cells. These changes comprise alterations known to be beneficial, but also adverse effects regarding AD were observed. Our results give an additional perspective of the complex link between MTX and AD, and suggest combining MTX with a lipid-altering diet compensating the adverse effects of MTX rather than using MTX alone to prevent or treat AD

The Casimir Effect in the Presence of a Minimal Length

Large extra dimensions lower the Planck scale to values soon accessible. Not only is the Planck scale the energy scale at which effects of modified gravity become important. The Planck length also acts as a minimal length in nature, providing a natural ultraviolet cutoff and a limit to the possible resolution of spacetime. In this paper we examine the influence of the minimal length on the Casimir energy between two plates.Comment: references and comment on the figure in section III adde

Gesamtwirtschaftliche Bewertung der Energiegewinnung aus Biomasse unter Berücksichtigung externer und makroökonomischer Effekte (Externe Effekte der Biomasse) : Endbericht

Weltweit herrscht weitgehend Konsens darüber, dass die mit einer nachhaltigen Entwicklung verbundenen Ziele u. a. einen verstärkten Einsatz erneuerbarer Energien bedingen. Betrachtet man in diesem Zusammenhang die Kosten und Potenziale der in Deutschland grundsätzlich nutzbaren erneuerbaren Energien, so zeigt sich, dass insbesondere die Biomasse bei „vertretbaren Kosten“ gegenüber heute einen deutlichen Mehrbeitrag zur Energieversorgung leisten könnte. Aus wohlfahrtstheoretischer bzw. gesamtwirtschaftlicher Sicht sind diese mit betriebswirtschaftlichem Kalkül ermittelten Kosten jedoch nicht ausreichend, um eine zuverlässige Beurteilung der Vorteilhaftigkeit von Energiesystemen zu ermöglichen. Die Bewertung der Förderwürdigkeit einer vermehrten Energiegewinnung aus Biomasse bedarf somit einer weitergehenden, auch gesamtwirtschaftliche Aspekte einbeziehenden Analyse. Eine derartige Bewertung wurde für die in Deutschland relevanten Bioenergiesysteme bisher allerdings noch nicht durchgeführt. Auch existiert noch keine allgemein anerkannte Standardmethodik zur gesamtwirtschaftlichen Bewertung von Energiesystemen. Das primäre Ziel dieser Studie ist es daher, die aus betriebswirtschaftlicher und gesamtwirtschaftlicher Sicht bedeutsamen Vor- und Nachteile einer verstärkten Energiegewinnung aus Biomasse in Deutschland zu ermitteln. Dazu muss zunächst eine geeignete Methodik entwickelt werden. Als Ergebnis der Untersuchung sollen schließlich Aussagen dazu getroffen werden, unter welchen Rand- und Rahmenbedingungen Möglichkeiten einer Energiebereitstellung aus biogenen Fest- und Flüssigbrennstoffen einerseits für den Betreiber, andererseits aber auch für die gesamte Volkswirtschaft am kostengünstigsten sind. Auch sollen Erkenntnisse dazu gewonnen werden, welche Entwicklungsmöglichkeiten die Bereitstellung von biogenen Flüssig- und Festbrennstoffen der heimischen Land- und Forstwirtschaft bieten. Die zur Erreichung dieser Zielsetzung durchgeführten Berechnungen haben schließlich gezeigt, dass die Energiegewinnung aus Biomasse zukünftig deutlich verstärkt in die Energieversorgung Deutschlands integriert werden sollte und dabei - unter Berücksichtigung quantifizierbarer externer und makroökonomischer Effekte - bereits heute zu erheblichen gesamtwirtschaftlichen Vorteilen wie z. B. der Stabilisierung des Agrar- und Forstsektors, positiven Nettobeschäftigungseffekten und einer reduzierten Abhängigkeit von Primärenergieimporten begleitet werden könnte. Darüber hinaus könnten durch Einsatz von Bioenergieträgern die Freisetzungen klimaschädlicher Gase und der Verbrauch erschöpflicher Energieressourcen signifikant reduziert werden. Da die gegenwärtigen energiewirtschaftlichen Rahmenbedingungen diese volkswirtschaftliche Vorteilhaftigkeit der Energiegewinnung aus Biomasse jedoch nicht in ausreichendem Maße in betriebswirtschaftliche Minderkosten übertragen, muss dies z. B. über den ergänzenden Einsatz von Fördermaßnahmen oder Besteuerung fossiler Energiesysteme erfolgen. Die maximale Erschließung der volkswirtschaftlichen Vorteile einer Energiegewinnung aus Biomasse setzt damit eine Ausgestaltung der aktuellen Förderinstrumente voraus

Building in China - Study trip of the faculty of Civil Engineering of the HTWG Konstanz 2008

Im März 2008 führte die Fakultät Bauingenieurwesen der HTWG Konstanz eine studentische Exkursion nach China durch. Auf dem Programm standen interessante Baustellen Shanghai, Nanjing, Zhenjiang und Beijing sowie der Besuch von Hochschulen. Der Exkursionsbericht beschreibt die besuchten Bauvorhaben und gibt persönliche Eindrücke der Exkursionsteilnehmer wieder.In March 2008 the faculty of civil engineering of the University of Applied Sciences Konstanz, Germany, conducted a study trip for students of civil engineering to China. Construction sites and universities in Shanghai, Nanjing, Zhenjiang and Beijing have been visited. The report describes the places seen and reflects the personal impressions of the participants

Laurent inversion

There are well-understood methods, going back to Givental and Hori--Vafa, that to a Fano toric complete intersection X associate a Laurent polynomial f that corresponds to X under mirror symmetry. We describe a technique for inverting this process, constructing the toric complete intersection X directly from its Laurent polynomial mirror f. We use this technique to construct a new four-dimensional Fano manifold

The EnMAP Observation Planning and Data Access for Scientific Users

EnMAP (Environmental Mapping and Analysis Program; www.enmap.org) is the first German imaging spectroscopy mission, to be launched in April 2022. After its Launch, Early Orbit and Commissioning Phase (6 months), the EnMAP mission will be available to the international user community for the data access and ordering process. EnMAP will be operated by the German Aerospace Center (DLR) covering all aspects relevant to assure successful mission operations. This comprises controlling and commanding the satellite using multi-mission infrastructures as well as observation planning, data reception, hyperspectral data processing including calibration, data archiving, data access and delivery, and providing web-interfaces to the international user community. This presentation will give an overview of EnMAP observation planning and data access concepts and outlines the data ordering workflow in particular for scientific users. The user can get access to EnMAP data using two different order options: On the one hand the user can submit future order requests through the EnMAP Data Access Portal (EDAP). The EDAP links to a set of functions for registered users that will support the international user community. This portal includes amongst others the proposal portal allowing submission of proposal for all scientific users responding to a Data Announcement of Opportunity (AO) and the Observation Request Portal providing planning support of observation requests and allowing submission of future orders. On the other hand, the already recorded data can be searched, processed and delivered based on catalogue from the archive through the EOWEB® GeoPortal. Although EnMAP is based on an open data policy and every type of user is in principle entitled to download data and request acquisitions, there will be different user categories to set acquisition priorities. The scientific (Cat-1) orders has higher priority and is requested to submit a proposal, which will undergo a scientific evaluation. The associated results will be presented by an interactive map supporting the establishment of a worldwide user network and guarantee the highest transparency of the proposal process. In the Observation Portal the user is able to submit future order requests by specifying following order parameters, such as the geographic area of interest (AOI) (between 74° North and 74° South), length of the AOI as a multiple of 30 km and up to 1000 km, the specification of the maximum allowable tilt angle of the satellite across the orbit (5° to 30°), the time span in which the acquisition should be performed and the option for time series and the number of data takes per months. To ensure acceptable illumination conditions, only images with sun zenith angle lower 60° will be considered. As for data acquisition EnMAP will be able to collect 5000 km along track and 30 km across track per day. The probability that the order will be included in the mission planning depends on the requirements for the observation as well as the specified priority and quota. Whether a specific data take is scheduled at the end depends on factors such as e.g. available data storage, cloud probabilities (e.g. historical and predicted cloud coverage) and, if requested by the user, sunglint probability (this is relevant for water products only). Users should make a request at least 25 hours before the scheduled recording to ensure the uplink. All data are available no later than 24 hours after collection for further processing into data products. The EnMAP ground segment will provide a range of standardized data products with different levels of processing of Level 1B, Level 1C and Level 2A based on archived Level 0 comprising extensive quicklooks and metadata. Due to required multiple processing options, each product is generated specifically for the order and delivered using FTPS (FTP with SSL) provided by multi-mission facilities