Operational performance of a PV generator feeding DC shunt and induction motors with MPPT

This paper presented the transient and operational behavior of a parallel Combination of DC Shunt Motor and IM fed by a photovoltaic generator at different solar irradiance levels. The maximum power point of current/voltage (I/V) characteristic of the PV generator was achieved for different solar intensities, by utilizing an open circuit voltage method. The nonlinear operational behavior of (I/V) characteristics of the PV generator at various solar intensities and the magnetization curve of the ferromagnetic material of the DC shunt motor were both modeled by high order polynomial mathematical expressions. The study investigated the response of the system at different solar irradiance levels and changing the torque loads for both motors and then following step change in solar intensity levels with fixed loading torques for both motors. All numerical simulations were executed using MATLAB software

Effect of treated wastewater irrigation on physiological and agronomic properties of beans Vicia faba

The current study investigated the effect of two doses (50%, and 100 %) of treated wastewater (TWW)on biometric and physiologic parameters of Vicia faba beansafter 40 days of exposure. Our data showed a decrease in shoots and roots length and weight in plants amended with TWW. Moreover, a significant decrease in Chlorophyll ‘a\u27, ‘b\u27 and carotene content was observedin plants irrigated with 100% of TWW. These findings provided new insights on TWW reuse which can cause different types of stress as it may affect the development of cultivated crops

Overview on Blood Transfusion-Transmitted Diseases

As it is important for the Blood transfusion to be extremely safe, some measures have to be taken long safeguarded the blood supply from the major transfusion transmissible diseases (TTIs).  The risk of transfusion-transmitted infection (TTI) rises with the number of donors exposed, and the effects of TTI are frequently more severe in immune compromised people. TTIs (hepatitis B virus [HBV], HIV, and hepatitis C virus [HCV]) are examples of typical transfusion-transmitted infectious agents. As a result of the gradual application of nucleic acid-amplification technology (NAT) screening for HIV, HCV, and HBV, the residual risk of infected window-period donations has been minimized. Nonetheless, infections emerge far more frequently than is commonly acknowledged, needing ongoing surveillance and individual assessment of transfusion-associated risk. Although there is a constant need to monitor present dangers owing to established TTI, the ongoing issues in blood safety are mostly related to surveillance for developing agents, as well as the creation of quick reaction systems when such agents are detected

Biallelic GRM7 variants cause epilepsy, microcephaly, and cerebral atrophy

Objective: Defects in ion channels and neurotransmitter receptors are implicated in developmental and epileptic encephalopathy (DEE). Metabotropic glutamate receptor 7 (mGluR7), encoded by GRM7, is a presynaptic G-protein-coupled glutamate receptor critical for synaptic transmission. We previously proposed GRM7 as a candidate disease gene in two families with neurodevelopmental disorders (NDDs). One additional family has been published since. Here, we describe three additional families with GRM7 biallelic variants and deeply characterize the associated clinical neurological and electrophysiological phenotype and molecular data in 11 affected individuals from six unrelated families. Methods: Exome sequencing and family-based rare variant analyses on a cohort of 220 consanguineous families with NDDs revealed three families with GRM7 biallelic variants; three additional families were identified through literature search and collaboration with a clinical molecular laboratory. Results: We compared the observed clinical features and variants of 11 affected individuals from the six unrelated families. Identified novel deleterious variants included two homozygous missense variants (c.2671G>A:p.Glu891Lys and c.1973G>A:p.Arg685Gln) and one homozygous stop-gain variant (c.1975C>T:p.Arg659Ter). Developmental delay, neonatal- or infantile-onset epilepsy, and microcephaly were universal. Three individuals had hypothalamic–pituitary–axis dysfunction without pituitary structural abnormality. Neuroimaging showed cerebral atrophy and hypomyelination in a majority of cases. Two siblings demonstrated progressive loss of myelination by 2 years in both and an acquired microcephaly pattern in one. Five individuals died in early or late childhood. Conclusion: Detailed clinical characterization of 11 individuals from six unrelated families demonstrates that rare biallelic GRM7 pathogenic variants can cause DEEs, microcephaly, hypomyelination, and cerebral atrophy. © 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association

MPV17â related mitochondrial DNA maintenance defect: New cases and review of clinical, biochemical, and molecular aspects

Mitochondrial DNA (mtDNA) maintenance defects are a group of diseases caused by deficiency of proteins involved in mtDNA synthesis, mitochondrial nucleotide supply, or mitochondrial dynamics. One of the mtDNA maintenance proteins is MPV17, which is a mitochondrial inner membrane protein involved in importing deoxynucleotides into the mitochondria. In 2006, pathogenic variants in MPV17 were first reported to cause infantileâ onset hepatocerebral mtDNA depletion syndrome and Navajo neurohepatopathy. To date, 75 individuals with MPV17â related mtDNA maintenance defect have been reported with 39 different MPV17 pathogenic variants. In this report, we present an additional 25 affected individuals with nine novel MPV17 pathogenic variants. We summarize the clinical features of all 100 affected individuals and review the total 48 MPV17 pathogenic variants. The vast majority of affected individuals presented with an earlyâ onset encephalohepatopathic disease characterized by hepatic and neurological manifestations, failure to thrive, lactic acidemia, and mtDNA depletion detected mainly in liver tissue. Rarely, MPV17 deficiency can cause a lateâ onset neuromyopathic disease characterized by myopathy and peripheral neuropathy with no or minimal liver involvement. Approximately half of the MPV17 pathogenic variants are missense. A genotype with biallelic missense variants, in particular homozygous p.R50Q, p.P98L, and p.R41Q, can carry a relatively better prognosis.MPV17 is a mitochondrial inner membrane protein involved in importing deoxynucleotides into the mitochondria. To date, 75 individuals with MPV17â related mitochondrial DNA (mtDNA) maintenance defect have been reported with 39 different MPV17 pathogenic variants. In this report, we present an additional 25 affected individuals with 9 novel MPV17 pathogenic variants. The vast majority of affected individuals presented with an earlyâ onset encephalohepatopathic disease. Rarely, MPV17 deficiency can cause a lateâ onset neuromyopathic disease with no or minimal liver involvement.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/142478/1/humu23387_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/142478/2/humu23387.pd

Mutations in EBF3 Disturb Transcriptional Profiles and Cause Intellectual Disability, Ataxia, and Facial Dysmorphism

From a GeneMatcher-enabled international collaboration, we identified ten individuals affected by intellectual disability, speech delay, ataxia, and facial dysmorphism and carrying a deleterious EBF3 variant detected by whole-exome sequencing. One 9-bp duplication and one splice-site, five missense, and two nonsense variants in EBF3 were found; the mutations occurred de novo in eight individuals, and the missense variant c.625C>T (p.Arg209Trp) was inherited by two affected siblings from their healthy mother, who is mosaic. EBF3 belongs to the early B cell factor family (also known as Olf, COE, or O/E) and is a transcription factor involved in neuronal differentiation and maturation. Structural assessment predicted that the five amino acid substitutions have damaging effects on DNA binding of EBF3. Transient expression of EBF3 mutant proteins in HEK293T cells revealed mislocalization of all but one mutant in the cytoplasm, as well as nuclear localization. By transactivation assays, all EBF3 mutants showed significantly reduced or no ability to activate transcription of the reporter gene CDKN1A, and in situ subcellular fractionation experiments demonstrated that EBF3 mutant proteins were less tightly associated with chromatin. Finally, in RNA-seq and ChIP-seq experiments, EBF3 acted as a transcriptional regulator, and mutant EBF3 had reduced genome-wide DNA binding and gene-regulatory activity. Our findings demonstrate that variants disrupting EBF3-mediated transcriptional regulation cause intellectual disability and developmental delay and are present in ∼0.1% of individuals with unexplained neurodevelopmental disorders

sPlotOpen - An environmentally balanced, open-access, global dataset of vegetation plots

Motivation Assessing biodiversity status and trends in plant communities is critical for understanding, quantifying and predicting the effects of global change on ecosystems. Vegetation plots record the occurrence or abundance of all plant species co-occurring within delimited local areas. This allows species absences to be inferred, information seldom provided by existing global plant datasets. Although many vegetation plots have been recorded, most are not available to the global research community. A recent initiative, called 'sPlot', compiled the first global vegetation plot database, and continues to grow and curate it. The sPlot database, however, is extremely unbalanced spatially and environmentally, and is not open-access. Here, we address both these issues by (a) resampling the vegetation plots using several environmental variables as sampling strata and (b) securing permission from data holders of 105 local-to-regional datasets to openly release data. We thus present sPlotOpen, the largest open-access dataset of vegetation plots ever released. sPlotOpen can be used to explore global diversity at the plant community level, as ground truth data in remote sensing applications, or as a baseline for biodiversity monitoring. Main types of variable contained Vegetation plots (n = 95,104) recording cover or abundance of naturally co-occurring vascular plant species within delimited areas. sPlotOpen contains three partially overlapping resampled datasets (c. 50,000 plots each), to be used as replicates in global analyses. Besides geographical location, date, plot size, biome, elevation, slope, aspect, vegetation type, naturalness, coverage of various vegetation layers, and source dataset, plot-level data also include community-weighted means and variances of 18 plant functional traits from the TRY Plant Trait Database. Spatial location and grain Global, 0.01-40,000 m(2). Time period and grain 1888-2015, recording dates. Major taxa and level of measurement 42,677 vascular plant taxa, plot-level records. Software format Three main matrices (.csv), relationally linked