Fragile X‐associated tremor/ataxia syndrome: Regional decrease of mitochondrial DNA copy number relates to clinical manifestations

Fragile X‐associated tremor/ataxia syndrome (FXTAS) is a late‐onset neurodegenerative disorder that appears in at least one‐third of adult carriers of a premutation (55‐200 CGG repeats) in the fragile X mental retardation 1 (FMR1) gene. Several studies have shown that mitochondrial dysfunction may play a central role in aging and also in neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, Huntington's disease as well as in FXTAS. It has been recently proposed that mtDNA copy number, measured by the number of mitochondrial genomes per nuclear genome (diploid), could be a useful biomarker of mitochondrial dysfunction. In order to elucidate the role of mtDNA variation in the pathogenesis of FXTAS, mtDNA copy number was quantified by digital droplet Polymerase chain reaction. In human brain samples, mtDNA levels were measured in the cerebellar vermis, dentate nucleus, parietal and temporal cortex, thalamus, caudate nucleus and hippocampus from a female FXTAS patient, a FMR1 premutation male carrier without FXTAS and from three male controls. The mtDNA copy number was further analyzed using this technology in dermal fibroblasts primary cultures derived from three FXTAS patients and three controls as well as in cortex and cerebellum of a CGG knock in FXTAS mice model. Finally, qPCR was carried out in human blood samples. Results indicate reduced mtDNA copy number in the specific brain region associated with disease progression in FXTAS patients, providing new insights into the role of mitochondrial dysfunction in the pathogenesis of FXTAS.     

New Immunomodulating Polyhydroxypregnane Glycosides from the Roots of Cynanchum otophyllum C.K.Schneid.

Seven new polyhydroxypregnane glycosides, named cynotophyllosides P–V, together with three known analogs were isolated from the roots of Cynanchum otophyllum C.K.Schneid . Their structures were elucidated by a variety of spectroscopic techniques, as well as acid‐catalyzed hydrolysis. All isolates were tested for their immunological activities in vitro against Con A‐ and LPS‐induced proliferation of mice splenocytes. Immunoenhancing (for 1 , 9 ) and immunosuppressive (for 2 ) activities were observed. Furthermore, cynotophylloside R ( 3 ) showed immunomodulatory as it enhanced the proliferation of splenocytes in low concentration and suppressed immune cells in concentration more than 1.0 μg/ml.     

Enhancing the Open‐Circuit Voltage of Perovskite Solar Cells by up to 120 mV Using π‐Extended Phosphoniumfluorene Electrolytes as Hole Blocking Layers

Four π‐extended phosphoniumfluorene electrolytes (π‐PFEs) are introduced as hole‐blocking layers (HBL) in inverted architecture planar perovskite solar cells with the structure of ITO/PEDOT:PSS/MAPbI₃/PCBM/HBL/Ag. The deep‐lying highest occupied molecular orbital energy level of the π‐PFEs effectively blocks holes, decreasing contact recombination. It is demonstrated that the incorporation of π‐PFEs introduces a dipole moment at the PCBM/Ag interface, resulting in significant enhancement of the built‐in potential of the device. This enhancement results in an increase in the open‐circuit voltage of the device by up to 120 mV, when compared to the commonly used bathocuproine HBL. The results are confirmed both experimentally and by numerical simulation. This work demonstrates that interfacial engineering of the transport layer/contact interface by small molecule electrolytes is a promising route to suppress nonradiative recombination in perovskite devices and compensates for a nonideal energetic alignment at the hole‐transport layer/perovskite interface.     

Metal–Organic Frameworks for Energy

Serious environmental problems, growing demand for energy, and the pursuit of environmental‐friendly, sustainable, and effective energy technologies to store and transform clean energy have all drawn great attention recently. As a part of the special issue “Energy Research in National Institute of Advanced Industrial Science and Technology (AIST)” this review systematically summarizes the research progress of metal–organic framework (MOF) composites and derivatives in energy applications, including catalytic CO oxidation, liquid‐phase chemical hydrogen storage, and electrochemical energy storage and conversion. Furthermore, the correlation between MOF‐based structures, synthetic strategies, and their corresponding performances is carefully discussed. The further scope and opportunities, expected improvements and challenges are also discussed. This review will not only benefit development of more feasible protocols to fabricate nanostructures for energy systems but also stimulate further interest in MOF composites and derivatives, for energy applications.     

Quantifying the Volumetric Performance Metrics of Supercapacitors

Nanostructured materials have greatly improved the performance of electrochemical energy storage devices because of the increased activity and surface area. However, nanomaterials (e.g., nanocarbons) normally possess low packing density, and thus occupy more space which restricts their suitability for making electrochemical devices as compact as possible. This has resulted in their low volumetric performance (capacitance, energy density, and power density), which is a practical obstacle for the application of nanomaterials in mobile and on‐board energy storage devices. While rating electrode materials for supercapacitors, their volumetric performance is equally important as the gravimetric metrics and more reliable in particular for systems with limited space. However, the adopted criteria for measuring the volumetric performance of supercapacitors vary in the literature. Identifying the appropriate performance criteria for the volumetric values will set a universal ground for valid comparison. Here, the authors discuss the rationale for quantifying the volumetric performance metrics of supercapacitors from the three progressive levels of materials, electrodes, and devices. It is hoped that these thoughts will be of value for the general community in energy storage research.     

Inter‐individual variation in colour patterns in noctuid moths characterizes long‐distance dispersers and agricultural pests

A high capacity for long‐distance dispersal is a key to success for species confronted with environmental heterogeneity, habitat modification, fragmentation and loss. However, dispersal capacity is difficult to quantify and therefore poorly known in most taxa. Here, we report on a test for an association of variation in dispersal capacity with variable colouration of noctuid moths. First, using data from 12 experienced lepidopterologists, we showed that despite variation among experts in average assessments, different species are consistently classified as having non‐variable, variable or highly variable colour patterns when assessed by different experts. We then compared the incidence of non‐resident species with high inter‐individual variation in colour patterns recorded on the isolated island Utklippan (n = 47), with that in a species pool of potential long‐distance dispersers from the nearest mainland (n = 295). Species with high inter‐individual colour pattern variation were over‐represented on the island compared with species having non‐variable colouration. This finding constitutes rare evidence from the wild of long‐distance dispersal, measured on a spatial scale relevant for moths when tracking habitats in fragmented and changing landscapes or when keeping pace with environmental challenges associated with climate change. Finally, we showed that Swedish noctuid moths classified as agricultural pests (n = 28) had more variable colour patterns compared with non‐pests (n = 368). The majority of agricultural pests were also recorded on the isolated island, an outcome that is indicative of pest species having high dispersal capacity. Data on colour pattern variation may thus offer a simple and cost‐effective proxy to estimate dispersal capacity and can also help identify potential pest species. Our findings are potentially useful when modelling and predicting population and range dynamics of species in spatiotemporally heterogeneous environments, with direct implications for conservation biology and pest management.     

Fine scale waterbody data improve prediction of waterbird occurrence despite coarse species data

While modelling habitat suitability and species distribution, ecologists must deal with issues related to the spatial resolution of species occurrence and environmental data. Indeed, given that the spatial resolution of species and environmental datasets range from centimeters to hundreds of kilometers, it underlines the importance of choosing the optimal combination of resolutions to achieve the highest possible modelling prediction accuracy. We evaluated how the spatial resolution of land cover/waterbody datasets (meters to 1 km) affect waterbird habitat suitability models based on atlas data (grid cell of 12 × 11 km). We hypothesized that the area, perimeter and number of waterbodies computed from high resolution datasets would explain distributions of waterbirds better because coarse resolution datasets omit small waterbodies affecting species occurrence. Specifically, we investigated which spatial resolution of waterbodies better explain the distribution of seven waterbirds nesting on ponds/lakes with areas ranging from 0.1 ha to hundreds of hectares. Our results show that the area and perimeter of waterbodies derived from high resolution datasets (raster data with 30 m resolution, vector data corresponding with map scale 1:10 000) explain the distribution of the waterbirds better than those calculated using less accurate datasets despite the coarse grain of the species data. Taking into account the spatial extent (global vs regional) of the datasets, we found the Global Inland Waterbody Dataset to be the most suitable for modelling distribution of waterbirds. In general, we recommend using land cover data of a resolution sufficient to capture the smallest patches of the habitat suitable for a given species’ presence for both fine and coarse grain habitat suitability and distribution modelling.