Minimal Kidney Disease Phenotype in Heterozygous Null Mice.

Background: Shroom family member 3 (SHROOM3) encodes an actin-associated protein that regulates epithelial morphology during development. Several genome-wide association studies (GWAS) have identified genetic variances primarily in the 5' region of SHROOM3, associated with chronic kidney disease (CKD) and poor transplant outcomes. These genetic variants are associated with alterations in Shroom3 expression.

Shroom3, a Gene Associated with CKD, Modulates Epithelial Recovery after AKI.

Background: Ischemia-induced AKI resulting in tubular damage can often progress to CKD and is a common cause of nephrology consultation. After renal tubular epithelial damage, molecular and cellular mechanisms are activated to repair and regenerate the damaged epithelium. If these mechanisms are impaired, AKI can progress to CKD.

Experimental evaluation of a linear angular momentum multiplexed radio link for moving platforms.

Linear angular momentum multiplexing (LAMM) has recently been proposed for high spectral-efficiency communications between moving platforms, such as between trains and ground infrastructure. We present performance results obtained from a scale experimental system comprising a 2 × 2 antenna system operating at 2.35 GHz.

Experimental evaluation of spectral efficiency from a circular array antenna producing a Laguerre-Gauss mode.

We present the four-dimensional volumetric electromagnetic field measurements (, , and frequency) of the complex radiated field produced by an 8-element circular antenna array. The array is designed to produce a Laguerre-Gauss (LG) mode = +1 over the frequency range of 9-10 GHz. We evaluate our findings in terms of far-field LG mode purity and spectral efficiency in terms of the quadrature amplitude modulation (QAM) modulation scheme that can be supported.

Linear angular momentum multiplexing-conceptualization and experimental evaluation with antenna arrays.

Linear angular momentum multiplexing is a new method for providing highly spectrally efficient short-range communication between a transmitter and receiver, where one may move at speed transverse to the propagation. Such applications include rail, vehicle and hyperloop transport systems communicating with fixed infrastructure on the ground. This paper describes how the scientific concept of linear angular momentum multiplexing evolves from orbital angular momentum multiplexing.

Experimental evaluation of 3D printed spiral phase plates for enabling an orbital angular momentum multiplexed radio system.

This paper evaluates the performance of three-dimensionally (3D) printed spiral phase plates (SPPs) for enabling an orbital angular momentum (OAM) multiplexed radio system. The design and realization of the SPPs by means of additive manufacturing exploiting a high-permittivity material is described. Modes 1 and 2 SPPs are then evaluated at 15 GHz in terms of 3D complex radiation pattern, mode purity and beam collimation by means of a 3D printed dielectric lens.

Bi-allelic Mutations in NADSYN1 Cause Multiple Organ Defects and Expand the Genotypic Spectrum of Congenital NAD Deficiency Disorders.

Birth defects occur in up to 3% of all live births and are the leading cause of infant death. Here we present five individuals from four unrelated families, individuals who share similar phenotypes with disease-causal bi-allelic variants in NADSYN1, encoding NAD synthetase 1, the final enzyme of the nicotinamide adenine dinucleotide (NAD) de novo synthesis pathway. Defects range from the isolated absence of both kidneys to multiple malformations of the vertebrae, heart, limbs, and kidney, and no affected individual survived for more than three months postnatally.

Performance analysis of an orbital angular momentum multiplexed amplify-and-forward radio relay chain with inter-modal crosstalk.

The end-to-end spectral efficiency and bit error rate (BER) of an amplify-and-forward (AF) radio relay chain employing orbital angular momentum (OAM) multiplexing is presented. The inherent divergence of a beam carrying OAM is overcome by means of a lens. Modelled and measured inter-modal crosstalk levels are incorporated into the analysis.

Sapropterin Treatment Prevents Congenital Heart Defects Induced by Pregestational Diabetes Mellitus in Mice.

Background Tetrahydrobiopterin is a cofactor of endothelial NO synthase ( eNOS ), which is critical to embryonic heart development. We aimed to study the effects of sapropterin (Kuvan), an orally active synthetic form of tetrahydrobiopterin on eNOS uncoupling and congenital heart defects ( CHD s) induced by pregestational diabetes mellitus in mice. Methods and Results Adult female mice were induced to pregestational diabetes mellitus by streptozotocin and bred with normal male mice to produce offspring.

Probing microwave fields and enabling in-situ experiments in a transmission electron microscope.

A technique is presented whereby the performance of a microwave device is evaluated by mapping local field distributions using Lorentz transmission electron microscopy (L-TEM). We demonstrate the method by measuring the polarisation state of the electromagnetic fields produced by a microstrip waveguide as a function of its gigahertz operating frequency. The forward and backward propagating electromagnetic fields produced by the waveguide, in a specimen-free experiment, exert Lorentz forces on the propagating electron beam.

Developmental Origins for Kidney Disease Due to Shroom3 Deficiency.

CKD is a significant health concern with an underlying genetic component. Multiple genome-wide association studies (GWASs) strongly associated CKD with the shroom family member 3 (SHROOM3) gene, which encodes an actin-associated protein important in epithelial morphogenesis. However, the role of SHROOM3 in kidney development and function is virtually unknown.

Rac1 Signaling Is Required for Anterior Second Heart Field Cellular Organization and Cardiac Outflow Tract Development.

Background: The small GTPase Rac1 regulates diverse cellular functions, including both apicobasal and planar cell polarity pathways; however, its role in cardiac outflow tract (OFT) development remains unknown. In the present study, we aimed to examine the role of Rac1 in the anterior second heart field (SHF) splanchnic mesoderm and subsequent OFT development during heart morphogenesis.

Methods And Results: Using the Cre/loxP system, mice with an anterior SHF-specific deletion of Rac1 (Rac1(SHF)) were generated.

Simulated diabetic ketoacidosis therapy in vitro elicits brain cell swelling via sodium-hydrogen exchange and anion transport.

A common complication of type 1 diabetes mellitus is diabetic ketoacidosis (DKA), a state of severe insulin deficiency. A potentially harmful consequence of DKA therapy in children is cerebral edema (DKA-CE); however, the mechanisms of therapy-induced DKA-CE are unknown. Our aims were to identify the DKA treatment factors and membrane mechanisms that might contribute specifically to brain cell swelling.

Understanding early organogenesis using a simplified in situ hybridization protocol in Xenopus.

Organogenesis is the study of how organs are specified and then acquire their specific shape and functions during development. The Xenopuslaevis embryo is very useful for studying organogenesis because their large size makes them very suitable for identifying organs at the earliest steps in organogenesis. At this time, the primary method used for identifying a specific organ or primordium is whole mount in situ hybridization with labeled antisense RNA probes specific to a gene that is expressed in the organ of interest.

Nanoantenna enhancement for telecom-wavelength superconducting single photon detectors.

Superconducting nanowire single photon detectors are rapidly emerging as a key infrared photon-counting technology. Two front-side-coupled silver dipole nanoantennas, simulated to have resonances at 1480 and 1525 nm, were fabricated in a two-step process. An enhancement of 50 to 130% in the system detection efficiency was observed when illuminating the antennas.

Hybridization of optical plasmonics with terahertz metamaterials to create multi-spectral filters.

Multi-spectral imaging systems typically require the cumbersome integration of disparate filtering materials in order to work simultaneously in multiple spectral regions. We show for the first time how a single nano-patterned metal film can be used to filter multi-spectral content from the visible, near infrared and terahertz bands by hybridizing plasmonics and metamaterials. Plasmonic structures are well-suited to the visible band owing to the resonant dielectric properties of metals, whereas metamaterials are preferable at terahertz frequencies where metal conductivity is high.

Expression of Ski can act as a negative feedback mechanism on retinoic acid signaling.

Background: Retinoic acid signaling is essential for many aspects of early development in vertebrates. To control the levels of signaling, several retinoic acid target genes have been identified that act to suppress retinoic acid signaling in a negative feedback loop. The nuclear protein Ski has been extensively studied for its ability to suppress transforming growth factor-beta (TGF-β) signaling but has also been implicated in the repression of retinoic acid signaling.

Narrowband multispectral filter set for visible band.

We design, fabricate and characterise a narrowband Fabry-Pérot multispectral filter set for the visible range (400-750 nm) that is suitable for integration onto complementary-metal oxide-semiconductor image sensors. We reduce the fabrication steps by fixing the physical cavity length and altering the effective optical length instead. Using electron-beam lithography, a sub-wavelength hole array is patterned in a silicon nitride cavity layer, backfilled with poly(methyl methacrylate), and bounded by aluminium mirrors to create 23 filters with full-width half-maximums of 22-46 nm.

Retinoic acid is a key regulatory switch determining the difference between lung and thyroid fates in Xenopus laevis.

Background: The lung and thyroid are derived from the anterior endoderm. Retinoic acid and Fgf signalling are known to be essential for development of the lung in mouse but little is known on how the lung and thyroid are specified in Xenopus.

Results: If either retinoic acid or Fgf signalling is inhibited, there is no differentiation of the lung as assayed by expression of sftpb.

Fgf is required to regulate anterior-posterior patterning in the Xenopus lateral plate mesoderm.

Given that the lateral plate mesoderm (LPM) gives rise to the cardiovascular system, identifying the cascade of signalling events that subdivides the LPM into distinct regions during development is an important question. Retinoic acid (RA) is known to be necessary for establishing the expression boundaries of important transcription factors that demarcate distinct regions along the anterior posterior axis of the LPM. Here, we demonstrate that fibroblast growth factor (Fgf) signalling is also necessary for regulating the expression domains of the same transcription factors (nkx2.

Postoperative acquired coagulopathy: a pilot study to determine the impact on clinical and economic outcomes.

Study Objective: To characterize the clinical factors associated with postoperative acquired coagulopathy, and to estimate the economic impact of resources used to treat postoperative patients with this coagulopathy compared with postoperative patients who did not develop the coagulopathy.

Design: Case-control study.

Setting: Academically affiliated public hospital and level I trauma referral center.

Direct activation of Shroom3 transcription by Pitx proteins drives epithelial morphogenesis in the developing gut.

Individual cell shape changes are essential for epithelial morphogenesis. A transcriptional network for epithelial cell shape change is emerging in Drosophila, but this area remains largely unexplored in vertebrates. The distinction is important as so far, key downstream effectors of cell shape change in Drosophila appear not to be conserved.