Tumor suppressive role of the antimicrobial lectin REG3A targeting the O -GlcNAc glycosylation pathway.

Background And Aims: Antimicrobial proteins of the regenerating family member 3 alpha (REG3A) family provide a first line of protection against infections and transformed cells. Their expression is inducible by inflammation, which makes their role in cancer biology less clear since an immune-inflammatory context may preexist or coexist with cancer, as occurs in HCC. The aim of this study is to clarify the role of REG3A in liver carcinogenesis and to determine whether its carbohydrate-binding functions are involved.

Helical coil design with controlled dispersion for bunching enhancement of protons generated by the target normal sheath acceleration.

The quality of the proton beam produced by target normal sheath acceleration (TNSA) with high-power lasers can be significantly improved with the use of helical coils. While they showed promising results in terms of focusing, their performances in terms of the of cut-off energy and bunching stay limited due to the dispersive nature of helical coils. A new scheme of helical coil with a tube surrounding the helix is introduced, and the first numerical simulations and an analytical model show a possibility of a drastic reduction of the current pulse dispersion for the parameters of high-power-laser facilities.

[Memories of the 13-November 2015 terrorist attacks: what discourse analysis can teach us].

The Programme 13-Novembre aims to analyze the individual and collective memory of the terrorist attacks of November 13, 2015. At its heart is the Étude 1000, which is to gather the same 1000 people in audiovisual interviews four times in 10 years. Having the transcripts at our disposal, we choose here to show the importance of discourse analysis by recalling its theoretical foundations, to present one of the tools allowing this statistical analysis, the Correspondence Factor Analysis, and to use it to analyze the sub-corpus of interviews conducted at a distance from the Paris events, with 76 inhabitants of the Metz region.

Antimicrobial protein REG3A regulates glucose homeostasis and insulin resistance in obese diabetic mice.

Innate immune mediators of pathogen clearance, including the secreted C-type lectins REG3 of the antimicrobial peptide (AMP) family, are known to be involved in the regulation of tissue repair and homeostasis. Their role in metabolic homeostasis remains unknown. Here we show that an increase in human REG3A improves glucose and lipid homeostasis in nutritional and genetic mouse models of obesity and type 2 diabetes.

Molecular and clinical descriptions of patients with GABA receptor gene variants (GABRA1, GABRB2, GABRB3, GABRG2): A cohort study, review of literature, and genotype-phenotype correlation.

Objective: γ-Aminobutyric acid (GABA) -receptor subunit variants have recently been associated with neurodevelopmental disorders and/or epilepsy. The phenotype linked with each gene is becoming better known. Because of the common molecular structure and physiological role of these phenotypes, it seemed interesting to describe a putative phenotype associated with GABA -receptor-related disorders as a whole and seek possible genotype-phenotype correlations.

Characterization of Optimized Ternary PLA/PHB/Organoclay Composites Processed through Fused Filament Fabrication and Injection Molding.

The aim of this study was to investigate the structure-properties relationship of ternary blends of polylactide/polyhydroxybutyrate (PLA/PHB)/organo-modified layered silicate (OMLS). Morphological, thermal, rheological, and mechanical characterizations were performed to understand the influence of OMLS on PLA/PHB (70/30 wt%) formulations optimized through modifications with an epoxy-based chain extender, the use of a plasticizer, as well as the influence of the type of processing route: injection molding or fused filament fabrication. The addition of OMLS allowed the blend compatibility to be improved, with the appearance of a single melting peak on DSC thermograms at 146 °C, as well as the reduction in the size of the nodules for the injected molded specimens.

Chemical Recycling of Vacuum-Infused Thermoplastic Acrylate-Based Composites Reinforced by Basalt Fabrics.

The objective of this work was to compare the material recovered from different chemical recycling methodologies for thermoplastic acrylate-based composites reinforced by basalt fabrics and manufactured by vacuum infusion. Recycling was done via chemical dissolution with a preselected adapted solvent. The main goal of the study was to recover undamaged basalt fabrics in order to reuse them as reinforcements for "second-generation" composites.

Mass Spectrometry-Based Proteomics Reveal Alcohol Dehydrogenase 1B as a Blood Biomarker Candidate to Monitor Acetaminophen-Induced Liver Injury.

Acute liver injury (ALI) is a severe disorder resulting from excessive hepatocyte cell death, and frequently caused by acetaminophen intoxication. Clinical management of ALI progression is hampered by the dearth of blood biomarkers available. In this study, a bioinformatics workflow was developed to screen omics databases and identify potential biomarkers for hepatocyte cell death.

Novel partial loss-of-function variants in the tyrosyl-tRNA synthetase 1 (YARS1) gene involved in multisystem disease.

Cytoplasmic aminoacyl-tRNA synthetases (ARSs) are emerging as a cause of numerous rare inherited diseases. Recently, biallelic variants in tyrosyl-tRNA synthetase 1 (YARS1) have been described in ten patients of three families with multi-systemic disease (failure to thrive, developmental delay, liver dysfunction, and lung cysts). Here, we report an additional subject with overlapping clinical findings, heterozygous for two novel variants in tyrosyl-tRNA synthetase 1 (NM_003680.

Colchicine for community-treated patients with COVID-19 (COLCORONA): a phase 3, randomised, double-blinded, adaptive, placebo-controlled, multicentre trial.

Background: Evidence suggests a role for excessive inflammation in COVID-19 complications. Colchicine is an oral anti-inflammatory medication beneficial in gout, pericarditis, and coronary disease. We aimed to investigate the effect of colchicine on the composite of COVID-19-related death or hospital admission.

Development of Bionanocomposites Based on Poly(3-Hydroxybutyrate-co-3-Hydroxyvalerate)/PolylActide Blends Reinforced with Cloisite 30B.

In the present study, poly(3-hydroxybuturate-co-3-hydroxyvalerate) (PHBV) and plasticized polylactide acid (PLA) blends were processed by melt extrusion with different weight ratio (up to 20 wt.% of PHBV). Bionanocomposites were obtained through the incorporation of an organomodified montmorillonite (C30B) at 3 wt.

Novel Extruded Starch-Beet Pulp Composites for Packaging Foams.

This article concerns the elaboration and the characterization of a novel biobased potato starch-beet pulp composite for packaging applications as cushion foams. A twin-screw extruder was used to elaborate composite foams. SEM observations of these materials were conducted, and thermomechanical properties were studied in terms of thermal transitions (TGA, DSC) and viscoelastic properties (DMA).

New Biosourced Flame Retardant Agents Based on Gallic and Ellagic Acids for Epoxy Resins.

The aim of this work was an investigation of the ability of gallic (GA) and ellagic (EA) acids, which are phenolic compounds encountered in various plants, to act as flame retardants (FRs) for epoxy resins. In order to improve their fireproofing properties, GA and EA were treated with boric acid (to obtain gallic acid derivatives (GAD) and ellagic acid derivatives (EAD)) to introduce borate ester moieties. Thermogravimetric analysis (TGA) highlighted the good charring ability of GA and EA, which was enhanced by boration.

Tannin Gels and Their Carbon Derivatives: A Review.

Tannins are one of the most natural, non-toxic, and highly reactive aromatic biomolecules classified as polyphenols. The reactive phenolic compounds present in their chemical structure can be an alternative precursor for the preparation of several polymeric materials for applications in distinct industries: adhesives and coatings, leather tanning, wood protection, wine manufacture, animal feed industries, and recently also in the production of new porous materials (i.e.

Clinical study of 19 patients with SCN8A-related epilepsy: Two modes of onset regarding EEG and seizures.

Objective: To describe the mode of onset of SCN8A-related severe epilepsy in order to facilitate early recognition, and eventually early treatment with sodium channel blockers.

Methods: We reviewed the phenotype of patients carrying a mutation in the SCN8A gene, among a multicentric cohort of 638 patients prospectively followed by several pediatric neurologists. We focused on the way clinicians made the diagnosis of epileptic encephalopathy, the very first symptoms, electroencephalography (EEG) findings, and seizure types.

IQSEC2-related encephalopathy in males and females: a comparative study including 37 novel patients.

Purpose: Variants in IQSEC2, escaping X inactivation, cause X-linked intellectual disability with frequent epilepsy in males and females. We aimed to investigate sex-specific differences.

Methods: We collected the data of 37 unpublished patients (18 males and 19 females) with IQSEC2 pathogenic variants and 5 individuals with variants of unknown significance and reviewed published variants.

Loss-of-Function Mutations in UNC45A Cause a Syndrome Associating Cholestasis, Diarrhea, Impaired Hearing, and Bone Fragility.

Despite the rapid discovery of genes for rare genetic disorders, we continue to encounter individuals presenting with syndromic manifestations. Here, we have studied four affected people in three families presenting with cholestasis, congenital diarrhea, impaired hearing, and bone fragility. Whole-exome sequencing of all affected individuals and their parents identified biallelic mutations in Unc-45 Myosin Chaperone A (UNC45A) as a likely driver for this disorder.

Sodium alginate adhesives as binders in wood fibers/textile waste fibers biocomposites for building insulation.

Alginate derived from seaweed is a natural polysaccharide able to form stable gel through carbohydrate functional groups largely used in the food and pharmaceutical industry. This article deals with the use of sodium alginate as an adhesive binder for wood fibres/textile waste fibres biocomposites. Several aldehyde-based crosslinking agents (glyoxal, glutaraldehyde) were compared for various wood/textile waste ratios (100/0, 50/50, 60/40, 70/30 and 0/100 in weight).

Missense mutation of TTC7A mimicking tricho-hepato-enteric (SD/THE) syndrome in a patient with very-early onset inflammatory bowel disease.

Tricho-hepato-enteric syndrome (SD/THE) and Multiple intestinal atresia with combined immune deficiency (MIA-CID) are autosomal recessive disorders that present immunological and gastrointestinal features. There are two different phenotypes of patients with TTC7A mutations: the severe form, caused by null mutations and leading to the classical MIA-CID; and the mild form, caused by missense mutations and leading to predominant features of VEO-IBD, less severe immunological involvement and hair abnormalities. We expand the knowledge about TTC7A deficiency, describing a patient with the mild phenotype of TTC7A deficiency but presenting overlapping features of SD/THE and MIA-CID: intestinal atresia and inflammatory bowel disease evocative of MIA-CID, but also dental abnormalities, huge forehead, liver abnormalities, autoimmune thyroiditis and hypogammaglobulinemia, evocative of SD/THE.

[NISCH syndrome, a rare cause of neonatal cholestasis: A case report].

NISCH syndrome is a rare autosomal recessive disease. It is characterized by scalp hypotrichosis, scarring alopecia, ichthyosis, and neonatal sclerosing cholangitis. It is caused by mutations in the CLDN1 gene encoding the claudin-1 protein, which is located at tight junctions.

Enteric Delivery of Regenerating Family Member 3 alpha Alters the Intestinal Microbiota and Controls Inflammation in Mice With Colitis.

Background & Aims: Paneth cell dysfunction causes deficiencies in intestinal C-type lectins and antimicrobial peptides, which leads to dysbiosis of the intestinal microbiota, alters the mucosal barrier, and promotes development of inflammatory bowel diseases. We investigated whether transgenic (TG) expression of the human regenerating family member 3 alpha gene (REG3A) alters the fecal microbiota and affects development of colitis in mice.

Methods: We performed studies with C57BL/6 mice that express human regenerating family member 3 alpha (hREG3A) in hepatocytes, via the albumin gene promoter.