Frontotemporal Dementia Caused by CHMP2B Mutations

CHMP2B mutations are a rare cause of autosomal dominant frontotemporal dementia (FTD). The best studied example is frontotemporal dementia linked to chromosome 3 (FTD-3) which occurs in a large Danish family, with a further CHMP2B mutation identified in an unrelated Belgian familial FTD patient. These mutations lead to C-terminal truncations of the CHMP2B protein and we will review recent advances in our understanding of the molecular effects of these mutant truncated proteins on vesicular fusion events within the endosome-lysosome and autophagy degradation pathways. We will also review the clinical features of FTD caused by CHMP2B truncation mutations as well as new brain imaging and neuropathological findings. Finally, we collate the current data on CHMP2B missense mutations, which have been reported in FTD and motor neuron disease

Multi-elemental speciation analysis of barley genotypes diering in tolerance to cadmium toxicity using SEC-ICP-MS and ESI-TOF-MS

Plants respond to Cd exposure by synthesizing heavy-metal-binding oligopeptides, called phytochelatins (PCs). These peptides reduce the activity of Cd2+ ions in the plant tissues by forming Cd chelates. The main objective of the present work was to develop an analytical technique, which allowed identication of the most prominent Cd species in plant tissue by SEC-ICP-MS and ESI-TOF-MS. An integrated part of the method development was to test the hypothesis that dierential Cd tolerance between two barley genotypes was linked to dierences in Cd speciation. Only one fraction of Cd species, ranging from 7001800 Da, was detected in the shoots of both genotypes. In the roots, two additional fractions ranging from 29004600 and 670015 000 Da were found. The Cd-rich SEC fractions were heart-cut, de-salted and demetallized using reversed-phase chromatography (RPC), followed by ESI-MS-TOF to identify the ligands. Three dierent families of PCs, viz. (gGlu-Cys)n-Gly (PCn), (gGlu-Cys)n-Ser (iso-PCn) and Cys-(gGlu-Cys)n-Gly (des-gGlu-PCn), the last lacking the N-terminal amino acid, were identied. The PCs induced by Cd toxicity also bound several essential trace elements in plants, including Zn, Cu, and Ni, whereas no Mn species were detected. Zn, Cu and Ni-species were distributed between the 7001800 Da and 670015 000 Da fractions, whereas only Cd species were found in the 29004600 Da fraction dominated by PC3 ligands. Although the total tissue concentration of Cd was similar for the two species, the tolerant barley genotype synthesized signicantly more CdPC3 species with a high Cd specicity than the intolerant genotype, clearly indicating a correlation between Cd tolerance and the CdPC speciation

Layout optimisation algorithms and reliability assessment of wind farm for microgrid integration:A comprehensive review

Abstract The paper represents a comprehensive review of the wind farm layout and reliability assessment of the wind farm integrated electrical power system. The authors have done a review on the proliferation of renewable energy which raises the uncertainties in the electrical power system. The uncertainties including wind speed and wake effect are important to deal with when an isolated microgrid is considered. The scenario becomes vigilant when the wind farms are integrated with the main grid. Due to uncertainties, the study of reliability evaluation of a wind integrated power system would become significant to analyse the electrical power system behaviour effectively. So, the paper discusses the layout optimisation methods of wind turbines considering the uncertainty parameters, mainly the wake effect. In this regard, the different wake models and optimisation methods based on a single‐objective and multi‐objective functions are reviewed in detail with the proper comparisons. The paper serves as a better illustration of the competency of these optimisation methods on the optimal wind turbine location on a wind farm. Furthermore, the paper extends the view on the reliability and cost assessment, and reliability improvement techniques of the wind integrated power system. This article provides comprehensive information, yields an attractive and subsequent tool for research requirements for the researchers to design the wind farm layout, and assessed the reliability of a wind integrated power system

Recent Excavations and Discoveries

Recent Excavations and Discoverie