Refine
Document Type
- Article (3)
- Monograph/Edited Volume (1)
Is part of the Bibliography
- yes (4)
Keywords
- (Anti)aromaticity (1)
- Anisotropy effect (1)
- Farber disease (1)
- Franconia (1)
- Franken (1)
- Genisa (1)
- Geniza (1)
- Hemiporphyrazines (1)
- Jewish Studies (1)
- Jüdische Studien (1)
Quantitative Bioimaging to Investigate the Uptake of Mercury Species in Drosophila melanogaster
(2015)
The uptake of mercury species in the model organism Drosophila melanogaster was investigated by elemental bioimaging using laser ablation-inductively coupled plasma mass spectrometry (LA-ICPMS). The mercury distribution in Drosophila melanogaster was analyzed for the three species mercury(II) chloride, methylmercury chloride, and thimerosal after intoxication. A respective analytical method was developed and applied to the analysis of the entire Drosophila melanogaster first, before a particular focus was directed to the cerebral areas of larvae and adult flies. For quantification of mercury, matrix-matched standards based on gelatin were prepared. Challenges of spatially dissolved mercury determination, namely, strong evaporation issues of the analytes and an inhomogeneous distribution of mercury in the standards due to interactions with cysteine containing proteins of the gelatin were successfully addressed by complexation with meso-2,3-dimercaptosuccinic acid (DMSA). No mercury was detected in the cerebral region for mercury(II) chloride, whereas both organic species showed the ability to cross the blood brain barrier. Quantitatively, the mercury level in the brain exceeded the fed concentration indicating mercury enrichment, which was approximately 3 times higher for methylmercury chloride than for thimerosal.
Farber disease (FD) is a rare lysosomal storage disorder resulting from acid ceramidase deficiency and subsequent ceramide accumulation. No treatments are clinically available and affected patients have a severely shortened lifespan. Due to the low incidence, the pathogenesis of FD is still poorly understood. Here, we report a novel acid ceramidase mutant mouse model that enables the study of pathogenic mechanisms of FD and ceramide accumulation. Asah1(tmEx1) mice were generated by deletion of the acid ceramidase signal peptide sequence. The effects on lysosomal targeting and activity of the enzyme were assessed. Ceramide and sphingomyelin levels were quantified by liquid chromatography tandem-mass spectrometry (LC-MS/MS) and disease manifestations in several organ systems were analyzed by histology and biochemistry. We show that deletion of the signal peptide sequence disrupts lysosomal targeting and enzyme activity, resulting in ceramide and sphingomyelin accumulation. The affected mice fail to thrive and die early. Histiocytic infiltrations were observed in many tissues, as well as lung inflammation, liver fibrosis, muscular disease manifestations and mild kidney injury. Our new mouse model mirrors human FD and thus offers further insights into the pathogenesis of this disease. In the future, it may also facilitate the development of urgently needed therapies.
The spatial magnetic properties (Through Space NMR Shieldings-TSNMRS) of a variety of porphyrins, hemiporphyrazines and tetraoxo[8]circulenes have been computed, visualized as Iso-chemical Shielding Surfaces (ICSS) of various size and direction, and were examined subject to the interplay of present (para)-diatropic ring currents [(anti)aromaticity] and influences on the latter property originating from the macrocyclic ring conformation, further annelation and partial to complete hydrogenation of aromatic ring moieties. Caution seems to be indicated when concluding from a single NICS parameter to present (para)diatropic ring currents [(anti)aromaticity]. (C) 2014 Elsevier Ltd. All rights reserved.
Genisa-Blätter III
(2020)
Ursprünglich sollten die „Genisa-Blätter III“ ein europäisches Projekt werden. Veröffentlicht werden sollten Quellen aus Genisot, die im vormals deutschsprachigen Raum gehoben wurden. Das Vorhaben scheiterte. Nunmehr sind im Band sieben Beiträge versammelt, in denen kenntnisreich Funde aus den Ablagen in Alsenz, Altenschönbach, Memmelsdorf, Reckendorf und Veitshöchheim besprochen werden. Der achte Aufsatz knüpft am internationalen Vorhaben an: Im Mittelpunkt des Artikels stehen drei Genisafunde aus Tschechien, genauer gesagt aus den beiden ostböhmischen Ortschaften Luže und Rychnov nad Kněžnou. Alle acht Beiträge gemeinsam verdeutlichen die inhaltliche Spannbreite von Genisot, die sich die Forschung zunutze machen kann: Sie reicht von Aspekten der Wirtschaft und des Rechts, über die Religion bis hin zum alltäglichen Leben und bietet jeweils eigene Geschichten im Spannungsfeld des möglichen Scheiterns.