Increased nuclear import characterizes aberrant nucleocytoplasmic transport in neurons from patients with spinocerebellar ataxia type 7.

Introduction: Spinocerebellar ataxia type 7 (SCA7) is an inherited neurodegenerative disorder characterized by cerebellar and retinal degeneration. SCA7 is caused by a CAG-polyglutamine repeat expansion in the ataxin-7 gene, which encodes a transcription factor protein that is a core component of the STAGA co-activator complex. As ataxin-7 protein regularly shuttles between the nucleus and the cytosol, we sought to test if polyglutamine-expanded ataxin-7 protein results in nuclear membrane abnormalities or defects in nucleocytoplasmic (N/C) transport.

Efficient Microwave Photon-to-Electron Conversion in a High-Impedance Quantum Circuit.

We demonstrate an efficient and continuous microwave photon-to-electron converter with large quantum efficiency (83%) and low dark current. These unique properties are enabled by the use of a high kinetic inductance disordered superconductor, granular aluminium, to enhance light-matter interaction and the coupling of microwave photons to electron tunneling processes. As a consequence of strong coupling, we observe both linear and nonlinear photon-assisted processes where two, three, and four photons are converted into a single electron at unprecedentedly low light intensities.

Activity of botulinum neurotoxin X and its structure when shielded by a non-toxic non-hemagglutinin protein.

Botulinum neurotoxins (BoNTs) are the most potent toxins known and are used to treat an increasing number of medical disorders. All BoNTs are naturally co-expressed with a protective partner protein (NTNH) with which they form a 300 kDa complex, to resist acidic and proteolytic attack from the digestive tract. We have previously identified a new botulinum neurotoxin serotype, BoNT/X, that has unique and therapeutically attractive properties.

Predictors and rate of survival after Out-of-Hospital Cardiac Arrest.

Background: Out-of-hospital cardiac arrest (OHCA) is a major public health concern and encloses a wide spectrum of causes. The purpose of this study is to assess predictors and rate of survival at hospital discharge and long-term in the setting of OHCA. The secondary endpoint is to compare OHCA-survival outcomes of presumed ischemic versus non ischemic cause.

HES6knockdown in human hematopoietic precursor cells reduces their in vivo engraftment potential and their capacity to differentiate into erythroid cells, B cells, T cells and plasmacytoid dendritic cells.

Hematopoiesis is driven by molecular mechanisms that induce differentiation and proliferation of hematopoietic stem cells and their progeny. This involves the activity of various transcription factors, such as members of the Hairy/Enhancer of Split (HES) family, and important roles for both HES1 and HES4 have been shown in normal and malignant hematopoiesis. Here, we investigated the role of HES6 in human hematopoiesis using in vitro and in vivo models.

Circadian organization of lipid landscape is perturbed in type 2 diabetic patients.

Lipid homeostasis in humans follows a diurnal pattern in muscle and pancreatic islets, altered upon metabolic dysregulation. We employ tandem and liquid-chromatography mass spectrometry to investigate daily regulation of lipid metabolism in subcutaneous white adipose tissue (SAT) and serum of type 2 diabetic (T2D) and non-diabetic (ND) human volunteers (n = 12). Around 8% of ≈440 lipid metabolites exhibit diurnal rhythmicity in serum and SAT from ND and T2D subjects.

Outcomes of Provisional Stenting With Versus Without Side Branch Intervention in Patients With Bifurcation Lesion-Related ST-Segment Elevation Myocardial Infarction.

To date, the best approach to coronary bifurcation lesion remains unsettled, and the parameters to guide side branch ballooning or stenting are not yet defined. This study aimed to compare the survival outcomes after provisional stenting with versus without side branch intervention. A cohort was conducted on 132 patients who underwent coronary angiography at Toulouse University Hospital for ST-segment elevation myocardial infarction with large culprit nonleft main coronary bifurcation lesion.

Tuning the Multiferroic Properties of BiFeO_{3} under Uniaxial Strain.

More than twenty years ago, multiferroic compounds combining in particular magnetism and ferroelectricity were rediscovered. Since then, BiFeO_{3} has emerged as the most outstanding multiferroic by combining at room temperature almost all the fundamental or applicative properties that may be desired: electroactive spin wave excitations called electromagnons, conductive domain walls, or a low band gap of interest for magnonic devices. All these properties have so far only been discontinuously strain engineered in thin films according to the lattice parameter imposed by the substrate.

Serotonin signaling regulates actomyosin contractility during morphogenesis in evolutionarily divergent lineages.

Serotonin is a neurotransmitter that signals through 5-HT receptors to control key functions in the nervous system. Serotonin receptors are also ubiquitously expressed in various organs and have been detected in embryos of different organisms. Potential morphogenetic functions of serotonin signaling have been proposed based on pharmacological studies but a mechanistic understanding is still lacking.

Phenotypic profiling of CD34 cells by advanced flow cytometry improves diagnosis of juvenile myelomonocytic leukemia.

Diagnostic criteria for juvenile myelomonocytic leukemia (JMML) are currently well defined, however in some patients diagnosis still remains a challenge. Flow cytometry is a well established tool for diagnosis and follow-up of hematological malignancies, nevertheless it is not routinely used for JMML diagnosis. Herewith, we characterized the CD34+ hematopoietic precursor cells collected from 31 children with JMML using a combination of standardized EuroFlow antibody panels to assess the ability to discriminate JMML cells from normal/reactive bone marrow cell as controls (n=29) or from cells of children with other hematological diseases mimicking JMML (n=9).

The impact of transsphenoidal surgery on pituitary function in patients with non-functioning macroadenomas.

Purpose: Transsphenoidal surgery for non-functioning pituitary adenomas (NFPAs) can alter pituitary function. We assessed the rates of improvement and deterioration of pituitary function by axis and searched for predictive factors of these outcomes.

Methods: We reviewed consecutive medical files from patients having had transsphenoidal surgery for NFPA between 2004 and 2018.

Experiences of Inter-Hospital Transfers (IHT) by Patients and Relatives during the COVID-19 Pandemic in France: A Qualitative Study.

Background: The first wave of the COVID-19 epidemic led to a rapid and unexpected saturation of the French ICU, forcing the health care system to adapt. Among other emergency measures, inter-hospital transfers were carried out.

Objective: To assess the psychological experience of patients and their relatives regarding inter-hospital transfers.

In situ uniaxial pressure cell for x-ray and neutron scattering experiments.

We present an in situ uniaxial pressure device optimized for small angle x-ray and neutron scattering experiments at low-temperatures and high magnetic fields. A stepper motor generates force, which is transmitted to the sample via a rod with an integrated transducer that continuously monitors the force. The device has been designed to generate forces up to 200 N in both compressive and tensile configurations, and a feedback control allows operating the system in a continuous-pressure mode as the temperature is changed.

Structure and activity of botulinum neurotoxin X.

Botulinum neurotoxins (BoNTs) are the most potent toxins known and are used to treat an increasing number of medical disorders. All BoNTs are naturally co-expressed with a protective partner protein (NTNH) with which they form a 300 kDa complex, to resist acidic and proteolytic attack from the digestive tract. We have previously identified a new botulinum neurotoxin serotype, BoNT/X, that has unique and therapeutically attractive properties.

[Influence of Comorbidity in Cognitive-Behavioral Treatment of Anxiety and Mood Disorders].

Objectives Anxiety and depression are the most prevalent disorders observed in health care services and are frequently comorbid with other disorders. Although Cognitive-Behavioral Therapy (CBT) has widely been shown efficacious to treat anxiety and mood disorders, studies that have focused on its effectiveness in the presence of comorbidity have been few and show conflicting results. Thus, the implications of the presence of comorbid disorders for treatment are still unknown.

Nematic-Fluctuation-Mediated Superconductivity Revealed by Anisotropic Strain in Ba(Fe_{1-x}Co_{x})_{2}As_{2}.

Anisotropic strain is an external field capable of selectively addressing the role of nematic fluctuations in promoting superconductivity. We demonstrate this using polarization-resolved elasto-Raman scattering by probing the evolution of nematic fluctuations under strain in the normal and superconducting state of the paradigmatic iron-based superconductor Ba(Fe_{1-x}Co_{x})_{2}As_{2}. In the parent compound BaFe_{2}As_{2} we observe a strain-induced suppression of the nematic susceptibility which follows the expected behavior of an Ising order parameter under a symmetry breaking field.

A contemporary baseline of Madagascar's coral assemblages: Reefs with high coral diversity, abundance, and function associated with marine protected areas.

Madagascar is a major hotspot of biodiversity in the Western Indian Ocean, but, as in many other regions, coral reefs surrounding the island confront large-scale disturbances and human-induced local stressors. Conservation actions have been implemented with encouraging results for fisheries, though their benefit on coral assemblages has never been rigorously addressed. In this context, we analyzed the multiscale spatial variation of the composition, generic richness, abundance, life history strategies, and cover of coral assemblages among 18 stations placed at three regions around the island.

The Euroflow PID Orientation Tube in the diagnostic workup of primary immunodeficiency: Daily practice performance in a tertiary university hospital.

Introduction: Multiparameter flow cytometry (FCM) immunophenotyping is an important tool in the diagnostic screening and classification of primary immunodeficiencies (PIDs). The EuroFlow Consortium recently developed the PID Orientation Tube (PIDOT) as a universal screening tool to identify lymphoid-PID in suspicious patients. Although PIDOT can identify different lymphoid-PIDs with high sensitivity, clinical validation in a broad spectrum of patients with suspicion of PID is missing.

Ferroportin Q248H mutation was not found to be protective against malaria and anemia in children under 5 years living in South Kivu/Democratic Republic of Congo, an endemic area of infection.

Background: Ferroportin (FPN) is known as an iron exporter and its effect on RBC iron could therefore hamper the growth of malaria parasites, since parasites are in need of iron. The aim of this study was to examine the prevalence of FPN Q248H in South Kivu/DRC and to evaluate its role in infected children and to explore its relationship with anemia.

Materials And Methods: We conducted a cross-sectional study in the health zone of Miti Murhesa in South Kivu/DRC.

Moderate to Severe Soft Tissue Diabetic Foot Infections: A Randomized, Controlled, Pilot Trial of Post-debridement Antibiotic Treatment for 10 versus 20 days.

Background: The optimal duration of antibiotic therapy for soft-tissue infections of the diabetic foot remains unknown.

Objective: We determine if antibiotic therapy after debridement for a short (10 days), compared with a long (20 days), duration for soft-tissue infections of the diabetic foot results in similar rates of clinical remission and adverse events (AE).

Summary Of Background Data: The optimal duration of systemic antibiotic therapy, after successful debridement, for soft tissue infections of diabetic patients is unknown.

Deciphering the Non-Coding RNA Landscape of Pediatric Acute Myeloid Leukemia.

Pediatric acute myeloid leukemia (pedAML) is a heterogeneous blood cancer that affects children. Although survival rates have significantly improved over the past few decades, 20-30% of children will succumb due to treatment-related toxicity or relapse. The molecular characterization of the leukemic stem cell, shown to be responsible for relapse, is needed to improve treatment options and survival.

Quality Assessment of a Large Multi-Center Flow Cytometric Dataset of Acute Myeloid Leukemia Patients-A EuroFlow Study.

Flowcytometric analysis allows for detailed identification and characterization of large numbers of cells in blood, bone marrow, and other body fluids and tissue samples and therefore contributes to the diagnostics of hematological malignancies. Novel data analysis tools allow for multidimensional analysis and comparison of patient samples with reference databases of normal, reactive, and/or leukemia/lymphoma patient samples. Building such reference databases requires strict quality assessment (QA) procedures.