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Far infrared (FIR) emission from the Earth's polar regions has become an area of increasing scientific interest and value. FIR emission is important for understanding Earth's radiative balance and improving global climate models, especially in rapidly changing Arctic conditions. Far‐infrared emission from Earth is not currently being monitored from space, except as part of broadband emission channels of Earth radiation budget measurements like those from the CERES project, and only limited measurements in the FIR spectrum exist. The Absolute Radiance Interferometer (ARI), developed as a prototype of the infrared spectrometer for CLARREO at the University of Wisconsin‐Madison, Space Science and Engineering Center, measures absolute spectrally resolved infrared (IR) radiance from 200 to 2,000 cm−1 (or 5–50 μm) at 0.5 cm−1 resolution with high accuracy (<0.1 K 3‐sigma brightness temperature at scene temperature). This instrument was taken into the field in Madison, Wisconsin, USA, during the winters of 2021 and 2022, where the weather can reach polar‐like conditions to measure high spectral resolution radiances of various sample types. Sample materials included water, snow, ice, evergreen leaves, dry grass, and sand, all characteristic of high latitude regions. Radiances collected from both a sky view and the sample view in clear‐sky conditions were used to retrieve FIR emissivity. This paper describes the ARI instrument configuration and capability for ground‐based measurements in the FIR region, and documents retrieved emissivities of various analyzed samples. The retrieved emissivity results are publicly available, and comparisons are made to simulated emissivity estimates. Key Points: Absolute Radiance Interferometer used to obtain high spectral resolution far infrared radiation measurements in semi‐controlled environmentsRadiation measurements of the sky and samples, including snow, water, ice, sand, and vegetation, allowed for retrieval of sample emissivityThe retrieved far infrared emissivity spectra and radiance measurements are available for model intercomparisons and other uses [ABSTRACT FROM AUTHOR]

High spectral resolution infrared sounders are an integral component of the global observing system and are used in a broad range of applications. This is enabled by their high accuracies which are ensured by rigorous calibration/validation activities. One of these activities is the post‐launch intercomparison with other high spectral resolution infrared sounders using simultaneous nadir overpasses (SNOs). This paper introduces a novel application of the previously developed SNO methodology by including time difference histogram symmetrization and a spatial sampling uncertainty. Where possible, radiometric measurement uncertainties are included and propagated through the statistics. Comparisons of Atmospheric Infrared Sounder (AIRS), METOP‐A/B/C Infrared Atmospheric Sounding Interferometer (IASI), and Cross‐track Infrared Sounder (CrIS) from 1 October 2019, to 1 October 2020, are analyzed. Results show AIRS and IASI differences as well as CrIS and AIRS differences are generally less than 0.4 K across the spectrum, and CrIS and IASI differences are generally less than 0.25 K. Comparison of the Suomi National Polar‐Orbiting Partnership and NOAA‐20 CrIS instruments via IASI and AIRS shows differences are generally less than 0.1 K across all bands and that the two CrIS instruments statistically agree within their radiometric uncertainties except for the narrow 2,370 cm−1 region where artifacts due to brightness temperature conversion are prone to occur. Plain Language Summary: One of the primary sources of information used to monitor Earth's weather patterns are satellite based meteorological instruments that measure radiation in the infrared region with high spectral resolution. Agencies worldwide use data from these types of instruments to provide input into weather prediction models. The long term data sets are also very valuable for multi‐decadal climate studies. To ensure the value of these data, the instruments need to be properly calibrated using external references. This paper details and expands upon the previously established methodology of one commonly used calibration technique—called SNOs. This method compares nadir‐viewing data from different instruments measuring radiation coming from the same mass of atmosphere in time and space. Introduced in this paper are two additions to the methodology: (a) a symmetrization of the time differences between when the instruments measure the specific air mass, and (b) an uncertainty estimate on the comparison based on the spatial coincidence of the measurements. Results from this methodology demonstration show how well the current suite of satellite‐based instrumentation measuring infrared radiation at high spectral resolution agree with each other. Key Points: Simultaneous nadir overpass comparisons include time difference symmetrization, spatial sampling uncertainty, and Cross‐track Infrared Sounder (CrIS) radiometric uncertaintyDifferences between Suomi National Polar‐Orbiting Partnership (SNPP) CrIS and NOAA‐20 CrIS are generally less than 0.1 K and within combined radiometric measurement uncertaintiesDifferences between Atmospheric Infrared Sounder (AIRS) and CrIS or AIRS and Infrared Atmospheric Sounding Interferometer (IASI) generally less than 0.4 K, differences between CrIS and IASI generally less than 0.25 K [ABSTRACT FROM AUTHOR]

We performed transcriptomic analyses on freshly frozen (n=21) and paraffin-embedded (n=35) gastrointestinal (GI) biopsies from children with and without acute acute GI graft-versus-host disease (GvHD) to study differential gene expressions. We identified 164 significant genes, 141 upregulated and 23 downregulated, in acute GvHD from freshy frozen biopsies. CHI3L1 was the top differentially expressed gene in acute GvHD, involved in macrophage recruitment and bacterial adhesion. Mitochondrial genes were among the top downregulated genes. Immune deconvolution identified a macrophage cellular signature. Weighted gene co-expression network analysis showed enrichment of genes in the ERK1/2 cascade. Transcriptome data from 206 ulcerative colitis (UC) patients were included to uncover genes and pathways shared between GvHD and UC. Comparison with the UC transcriptome showed both shared and distinct pathways. Both UC and GvHD transcriptomes shared an innate antimicrobial signature and FCγ1RA/CD64 was upregulated in both acute GvHD (log-fold increase 1.7, P=0.001) and UC. Upregulation of the ERK1/2 cascade pathway was specific to GvHD. We performed additional experiments to confirm transcriptomics. Firstly, we examined phosphorylation of ERK (pERK) by immunohistochemistry on GI biopsies (acute GvHD n=10, no GvHD n=10). pERK staining was increased in acute GvHD biopsies compared to biopsies without acute GvHD (P=0.001). Secondly, plasma CD64, measured by enzyme-linked immunsorbant assay (n=85) was elevated in acute GI GvHD (P<0.001) compared with those without and was elevated in GVHD compared with inflammatory bowel disease (n=47) (P<0.001), confirming the upregulated expression seen in the transcriptome.

The long-term outcomes of adults with Fanconi anaemia (FA) have improved with advances in haematopoietic stem cell transplantation (HSCT) and more detailed follow-up and screening guidelines. The phenotype of those who survive to adulthood may differ from the typical presentation of FA. We collected retrospective clinical data on adults with FA who received their care at the Cincinnati Children's Hospital Medical Center. In our final cohort of 52 patients, there were 29 females and 23 males, with median (range) age of 21 (18-37) years. Overall, 42 patients (81%) were alive at last follow-up. In all, 36 adults (69%) had undergone HSCT, including eight who had developed myelodysplasia or acute myeloid leukaemia. Eight (15%) developed squamous cell carcinoma. Endocrine complications were common, including hypothyroidism (42%), diabetes (10%), low body mass index (31%) and low bone mineral density (51%). The majority of adults with FA were employed (52%) or full-time students (13%). A significant subset of patients with FA are surviving into adulthood without requiring HSCT. Endocrine abnormalities and the development of solid tumours complicate adulthood. With improved survival outcomes following HSCT and more aggressive malignancy screening protocols, ongoing longitudinal analysis will be important to further characterise this cohort and the phenotype of untransplanted adults with FA., (© 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

TSPEAR variants cause autosomal recessive ectodermal dysplasia (ARED) 14. The function of TSPEAR is unknown. The clinical features, the mutation spectrum, and the underlying mechanisms of ARED14 are poorly understood. Combining data from new and previously published individuals established that ARED14 is primarily characterized by dental anomalies such as conical tooth cusps and hypodontia, like those seen in individuals with WNT10A -related odontoonychodermal dysplasia. AlphaFold-predicted structure-based analysis showed that most of the pathogenic TSPEAR missense variants likely destabilize the β-propeller of the protein. Analysis of 100000 Genomes Project (100KGP) data revealed multiple founder TSPEAR variants across different populations. Mutational and recombination clock analyses demonstrated that non-Finnish European founder variants likely originated around the end of the last ice age, a period of major climatic transition. Analysis of gnomAD data showed that the non-Finnish European population TSPEAR gene-carrier rate is ∼1/140, making it one of the commonest AREDs. Phylogenetic and AlphaFold structural analyses showed that TSPEAR is an ortholog of drosophila Closca , an extracellular matrix-dependent signaling regulator. We, therefore, hypothesized that TSPEAR could have a role in enamel knot, a structure that coordinates patterning of developing tooth cusps. Analysis of mouse single-cell RNA sequencing (scRNA-seq) data revealed highly restricted expression of Tspear in clusters representing enamel knots. A tspeara -/- ; tspearb -/- double-knockout zebrafish model recapitulated the clinical features of ARED14 and fin regeneration abnormalities of wnt10a knockout fish, thus suggesting interaction between tspear and wnt10a. In summary, we provide insights into the role of TSPEAR in ectodermal development and the evolutionary history, epidemiology, mechanisms, and consequences of its loss of function variants., Competing Interests: The authors declare no competing interests., (© 2023 The Author(s).)