Eight induced pluripotent stem cell lines (iPSCs) derived from two patients with Leukocyte adhesion deficiency Type I (LAD I) with mutations in the ITGB2 gene.

Leukocyte Adhesion Deficiency Type I (LAD I) is a rare inborn error of immunity caused by mutations in the ITGB2 gene coding for β2-integrin CD18 on the surface of leukocytes. Affected patients display severe clinical manifestations with life threatening infections and inflammatory complications due to an impaired ability of leukocytes to transmigrate from the blood vessel to the tissue. Here we describe the generation of eight induced pluripotent stem cell lines from two patients with LAD I and mutations in the ITGB2 gene.

Unravelling the amorphous structure and crystallization mechanism of GeTe phase change memory materials.

The reversible phase transitions in phase-change memory devices can switch on the order of nanoseconds, suggesting a close structural resemblance between the amorphous and crystalline phases. Despite this, the link between crystalline and amorphous tellurides is not fully understood nor quantified. Here we use in-situ high-temperature x-ray absorption spectroscopy (XAS) and theoretical calculations to quantify the amorphous structure of bulk and nanoscale GeTe.

Phase-Change Memory from Molecular Tellurides.

Phase-change memory (PCM) is an emerging memory technology based on the resistance contrast between the crystalline and amorphous states of a material. Further development and realization of PCM as a mainstream memory technology rely on innovative materials and inexpensive fabrication methods. Here, we propose a generalizable and scalable solution-processing approach to synthesize phase-change telluride inks in order to meet demands for high-throughput material screening, increased energy efficiency, and advanced device architectures.

Expression of a large coding sequence: Gene therapy vectors for Ataxia Telangiectasia.

Ataxia telangiectasia is a monogenetic disorder caused by mutations in the ATM gene. Its encoded protein kinase ATM plays a fundamental role in DNA repair of double strand breaks (DSBs). Impaired function of this kinase leads to a multisystemic disorder including immunodeficiency, progressive cerebellar degeneration, radiation sensitivity, dilated blood vessels, premature aging and a predisposition to cancer.

Colloidal Ternary Telluride Quantum Dots for Tunable Phase Change Optics in the Visible and Near-Infrared.

A structural change between amorphous and crystalline phase provides a basis for reliable and modular photonic and electronic devices, such as nonvolatile memory, beam steerers, solid-state reflective displays, or mid-IR antennas. In this paper, we leverage the benefits of liquid-based synthesis to access phase-change memory tellurides in the form of colloidally stable quantum dots. We report a library of ternary MGeTe colloids (where M is Sn, Bi, Pb, In, Co, Ag) and then showcase the phase, composition, and size tunability for Sn-Ge-Te quantum dots.

Environmental formaldehyde sensing at room temperature by smartphone-assisted and wearable plasmonic nanohybrids.

Formaldehyde is a toxic and carcinogenic indoor air pollutant. Promising for its routine detection are gas sensors based on localized surface plasmon resonance (LSPR). Such sensors trace analytes by converting tiny changes in the local dielectric environment into easily readable, optical signals.

Mass Mortality Caused by Highly Pathogenic Influenza A(H5N1) Virus in Sandwich Terns, the Netherlands, 2022.

We collected data on mass mortality in Sandwich terns (Thalasseus sandvicensis) during the 2022 breeding season in the Netherlands. Mortality was associated with at least 2 variants of highly pathogenic avian influenza A(H5N1) virus clade 2.3.

Influenza A virus infection instructs hematopoiesis to megakaryocyte-lineage output.

Respiratory tract infections are among the deadliest communicable diseases worldwide. Severe cases of viral lung infections are often associated with a cytokine storm and alternating platelet numbers. We report that hematopoietic stem and progenitor cells (HSPCs) sense a non-systemic influenza A virus (IAV) infection via inflammatory cytokines.

Acetone Sensing and Catalytic Conversion by Pd-Loaded SnO.

Noble metal additives are widely used to improve the performance of metal oxide gas sensors, most prominently with palladium on tin oxide. Here, we photodeposit different quantities of Pd (0-3 mol%) onto nanostructured SnO and determine their effect on sensing acetone, a critical tracer of lipolysis by breath analysis. We focus on understanding the effect of operating temperature on acetone sensing performance (sensitivity and response/recovery times) and its relationship to catalytic oxidation of acetone through a packed bed of such Pd-loaded SnO.

Forming megakaryocytes from murine-induced pluripotent stem cells by the inducible overexpression of supporting factors.

Background: Platelets are small anucleate cells that circulate in the blood in a resting state but can be activated by external cues. In case of need, platelets from blood donors can be transfused. As an alternative source, platelets can be produced from induced pluripotent stem cells (iPSCs); however, recovered numbers are low.

The StemCellFactory: A Modular System Integration for Automated Generation and Expansion of Human Induced Pluripotent Stem Cells.

While human induced pluripotent stem cells (hiPSCs) provide novel prospects for disease-modeling, the high phenotypic variability seen across different lines demands usage of large hiPSC cohorts to decipher the impact of individual genetic variants. Thus, a much higher grade of parallelization, and throughput in the production of hiPSCs is needed, which can only be achieved by implementing automated solutions for cell reprogramming, and hiPSC expansion. Here, we describe the StemCellFactory, an automated, modular platform covering the entire process of hiPSC production, ranging from adult human fibroblast expansion, Sendai virus-based reprogramming to automated isolation, and parallel expansion of hiPSC clones.

The lesser purple emperor butterfly, : from mimesis to biomimetics.

Until now, hues as dynamic as those adorning the Apatura emperor butterflies have never been encountered in the painting world. Unlike and unmatched by the chemical pigments traditionally found on the painter's palette, the emperor's wings are studded with strongly reflecting iridescent scales that are structured like those of the iconic Morpho butterflies. The scale ridges act as diffractive multilayers, giving rise to narrow-band reflectance spectra.

Signaling properties of murine MPL and MPL mutants after stimulation with thrombopoietin and romiplostim.

Thrombopoietin (THPO) and its receptor myeloproliferative leukemia virus oncogene (MPL) regulate hematopoietic stem cell (HSC) quiescence and maintenance, but also megakaryopoiesis. Thrombocytopenias or aplastic anemias can be treated today with THPO peptide mimetics (romiplostim) or small-molecule THPO receptor agonists (e.g.

Endothelial protein C receptor supports hematopoietic stem cell engraftment and expansion in Mpl-deficient mice.

Thrombopoietin (Thpo)/myeloproliferative leukemia virus oncogene (Mpl) signaling controls hematopoietic stem cell (HSC) self-renewal and quiescence; however, how these 2 seemingly opposing functions are controlled is not well understood. By transplantation of lentiviral-transduced hematopoietic cells in the Mpl-deficient mouse model, we addressed whether known or predicted Thpo target genes were able to rescue the Mpl-deficient phenotype of the mice. Among the tested genes, we identified endothelial protein C receptor (Epcr) to expand HSCs with the long-term (LT)-HSC surface phenotype in Mpl mice and to enable secondary transplantation of Mpl-deficient bone marrow (BM).

Sustained and regulated gene expression by Tet-inducible "all-in-one" retroviral vectors containing the HNRPA2B1-CBX3 UCOE.

Genetic modification of induced pluripotent stem (iPS) cells may be necessary for the generation of effector cells for cellular therapies. Hereby, it can be important to induce transgene expression at restricted and defined time windows, especially if it interferes with pluripotency or differentiation. To achieve this, inducible expression systems can be used such as the tetracycline-inducible retroviral vector system, however, retroviral expression can be subjected to epigenetic silencing or to position-effect variegation.

Increase in CO concentration could alter the response of to climate change.

Increasing CO concentration ([CO ]) is likely to affect future species distributions, in interaction with other climate change drivers. However, current modeling approaches still seldom consider interactions between climatic factors and the importance of these interactions therefore remains mostly unexplored. Here, we combined dendrochronological and modeling approaches to study the interactive effects of increasing [CO ] and temperature on the distribution of one of the main European liana species, .

Warm summers during the Younger Dryas cold reversal.

The Younger Dryas (YD) cold reversal interrupts the warming climate of the deglaciation with global climatic impacts. The sudden cooling is typically linked to an abrupt slowdown of the Atlantic Meridional Overturning Circulation (AMOC) in response to meltwater discharges from ice sheets. However, inconsistencies regarding the YD-response of European summer temperatures have cast doubt whether the concept provides a sufficient explanation.

Development of a paper-based lateral flow immunoassay for simultaneous detection of lipopolysaccharides of Salmonella serovars.

Lateral flow type detection is becoming interesting not only in regions with a poor medical infrastructure but also for practitioners in day-to-day clinical work or for veterinary control in case of possible epidemics. In this work, we describe the first steps of development of a multi-channel strip with potential internal calibration of multiparametric and colorimetric lateral flow assays for the simultaneous detection of the lipopolysaccharides (LPS) of Salmonella typhimurium (S. typhimurium) and Salmonella enteritidis (S.

Engineering Next-Generation BET-Independent MLV Vectors for Safer Gene Therapy.

Retroviral vectors have shown their curative potential in clinical trials correcting monogenetic disorders. However, therapeutic benefits were compromised due to vector-induced dysregulation of cellular genes and leukemia development in a subset of patients. Bromodomain and extraterminal domain (BET) proteins act as cellular cofactors that tether the murine leukemia virus (MLV) pre-integration complex to host chromatin via interaction with the MLV integrase (IN) and thereby define the typical gammaretroviral integration distribution.

Targeting expression to megakaryocytes and platelets by lineage-specific lentiviral vectors.

Unlabelled: Essentials Platelet phenotypes can be modified by lentiviral transduction of hematopoietic stem cells. Megakaryocyte-specific lentiviral vectors were tested in vitro and in vivo for restricted expression. The glycoprotein 6 vector expressed almost exclusively in megakaryocytes.

High-speed microscopy of continuously moving cell culture vessels.

We report a method of high-speed phase contrast and bright field microscopy which permits large cell culture vessels to be scanned at much higher speed (up to 30 times faster) than when conventional methods are used without compromising image quality. The object under investigation moves continuously and is captured using a flash illumination which creates an exposure time short enough to prevent motion blur. During the scan the object always stays in focus due to a novel hardware-autofocus system.

Anxiety modulates cognitive deficits in a perinatal glutathione deficit animal model of schizophrenia.

In this study, we investigated long-term repercussion of early glutathione deficit by l-buthionine-(S,R)-sulfoximine (BSO) injections as a rat model of schizophrenia. BSO rats were tested through various behavioral tasks requiring animals to take into account previously delivered information. We showed that relative to controls, BSO rats (1) were less active and more anxious in an Elevated Plus Maze test, allowing us to split them into two subgroups with high and low anxiety levels; (2) demonstrated normal abilities of behavioral flexibility tested with a rat-adapted version of the Wisconsin Card Sorting Test (WCST), with even higher abilities in anxious BSO rats suggesting reduced interference of previously acquired rules; (3) did not forage normally in radial arm mazes and mainly used clockwise strategies; (4) exhibited a lack of habituation during a startle response task; and (5) showed a normal prepulse inhibition of the startle response (PPI) and a normal conditioned taste aversion (CTA).

CD30 Receptor-Targeted Lentiviral Vectors for Human Induced Pluripotent Stem Cell-Specific Gene Modification.

Cultures of induced pluripotent stem cells (iPSCs) often contain cells of varying grades of pluripotency. We present novel lentiviral vectors targeted to the surface receptor CD30 (CD30-LV) to transfer genes into iPSCs that are truly pluripotent as demonstrated by marker gene expression. We demonstrate that CD30 expression is restricted to SSEA4(high) cells of human iPSC cultures and a human embryonic stem cell line.