Publications by authors named "Courtois G"

Article Synopsis
  • * A study examined 131 female patients with X-linked dominant incontinentia pigmenti (IP), finding that 36% produced autoantibodies against IFN-α and/or IFN-ω, significantly higher than age-matched controls.
  • * The presence of these autoantibodies is linked to an abnormally small thymus and predisposes patients to life-threatening viral infections, while those without these autoantibodies do not face the same risk.
View Article and Find Full Text PDF
Article Synopsis
  • Lower limb exoskeletons are gaining attention for post-stroke rehabilitation, often used alongside traditional physiotherapy during the subacute phase for gait training.
  • The main challenge in gait training is generating reference trajectories, which typically mimic the average walking patterns of healthy individuals.
  • This paper presents an online trajectory generation (OTG) algorithm that replicates the movement paths of therapists during rehabilitation tasks, with experimental results demonstrating its effectiveness at a 500µs cycle time.
View Article and Find Full Text PDF

Germanium (Ge) is increasingly used as a substrate for high-performance optoelectronics, photovoltaics, and electronic devices. These devices are usually grown on thick and rigid Ge substrates manufactured by classical wafering techniques. Nanomembranes (NMs) provide an alternative to this approach while offering wafer-scale lateral dimensions, weight reduction, waste limitation, and cost effectiveness.

View Article and Find Full Text PDF

Introduction: Microcytic anemias (MA) have frequent or rare etiologies. New discoveries in understanding and treatment of microcytic anemias need to be reviewed.

Areas Covered: Microcytic anemias with a focus on the most frequent causes and on monogenic diseases that are relevant for understanding biocellular mechanisms of MA.

View Article and Find Full Text PDF
Article Synopsis
  • Gestational choriocarcinoma (GC) is a serious tumor connected to hydatidiform moles, with NLRP7 being a key gene linked to its development and immune response.
  • *Research shows that NLRP7 can operate through different pathways, significantly influencing cancer cell survival and aggressive traits in tumor cells compared to non-tumor cells.
  • *Finding that NLRP7 plays a crucial role in GC growth suggests it could be a valuable target for new treatment strategies for resistant patients.
View Article and Find Full Text PDF
Article Synopsis
  • Researchers studied two proteins, RIPK3 and MLKL, that are important for a type of cell death called necroptosis, which can make diseases worse.
  • They found that when they removed RIPK3 and MLKL in lab models, the cells could not change into white fat cells, but they could still change into another type of fat cell called beige fat cells.
  • This means RIPK3 and MLKL have different jobs in making fat cells, and focusing on blocking MLKL might help treat obesity better.
View Article and Find Full Text PDF

Red blood cell production is negatively controlled by the rate of apoptosis at the stage of CFU-E/pro-erythroblast differentiation, depending on the balance between erythropoietin (EPO) levels and activation of the Fas/FasL pathway. At this stage, activation of transient caspases through depolarization via mitochondrial outer membrane permeabilization (MOMP) is also required for terminal erythroid differentiation. Molecular mechanisms regulating the differential levels of MOMP during differentiation and apoptosis, however, remain poorly understood.

View Article and Find Full Text PDF

For many years, clinicians have understood the advantages of listening with two ears compared with one. In addition to improved speech intelligibility in quiet, noisy, and reverberant environments, binaural versus monaural listening improves perceived sound quality and decreases the effort listeners must expend to understand a target voice of interest or to monitor a multitude of potential target voices. For most individuals with bilateral hearing impairment, the body of evidence collected across decades of research has also found that the provision of two compared with one hearing aid yields significant benefit for the user.

View Article and Find Full Text PDF

β-thalassemia major (β-TM) is an inherited hemoglobinopathy caused by a quantitative defect in the synthesis of β-globin chains of hemoglobin, leading to the accumulation of free a-globin chains that aggregate and cause ineffective erythropoiesis. We have previously demonstrated that terminal erythroid maturation requires a transient activation of caspase-3 and that the chaperone Heat Shock Protein 70 (HSP70) accumulates in the nucleus to protect GATA-1 transcription factor from caspase-3 cleavage. This nuclear accumulation of HSP70 is inhibited in human β-TM erythroblasts due to HSP70 sequestration in the cytoplasm by free a-globin chains, resulting in maturation arrest and apoptosis.

View Article and Find Full Text PDF

The auditory system allows the estimation of the distance to sound-emitting objects using multiple spatial cues. In virtual acoustics over headphones, a prerequisite to render auditory distance impression is sound externalization, which denotes the perception of synthesized stimuli outside of the head. Prior studies have found that listeners with mild-to-moderate hearing loss are able to perceive auditory distance and are sensitive to externalization.

View Article and Find Full Text PDF

Background & Aims: In non-alcoholic fatty liver disease (NAFLD), hepatocytes can undergo necroptosis: a regulated form of necrotic cell death mediated by the receptor-interacting protein kinase (RIPK) 1. Herein, we assessed the potential for RIPK1 and its downstream effector mixed lineage kinase domain-like protein (MLKL) to act as therapeutic targets and markers of activity in NAFLD.

Methods: C57/BL6J-mice were fed a normal chow diet or a high-fat diet (HFD).

View Article and Find Full Text PDF

Tryptophan as the precursor of several active compounds, including kynurenine and serotonin, is critical for numerous important metabolic functions. Enhanced tryptophan metabolism toward the kynurenine pathway has been associated with myelodysplastic syndromes (MDSs), which are preleukemic clonal diseases characterized by dysplastic bone marrow and cytopenias. Here, we reveal a fundamental role for tryptophan metabolized along the serotonin pathway in normal erythropoiesis and in the physiopathology of MDS-related anemia.

View Article and Find Full Text PDF

Diamond-Blackfan anemia (DBA) is a congenital erythroblastopenia that is characterized by a blockade in erythroid differentiation related to impaired ribosome biogenesis. DBA phenotype and genotype are highly heterogeneous. We have previously identified 2 in vitro erythroid cell growth phenotypes for primary CD34 cells from DBA patients and following short hairpin RNA knockdown of RPS19, RPL5, and RPL11 expression in normal human CD34 cells.

View Article and Find Full Text PDF

The nuclear factor κB (NF-κB) signaling pathway ubiquitously controls cell growth and survival in basic conditions as well as rapid resetting of cellular functions following environment changes or pathogenic insults. Moreover, its deregulation is frequently observed during cell transformation, chronic inflammation or autoimmunity. Understanding how it is properly regulated therefore is a prerequisite to managing these adverse situations.

View Article and Find Full Text PDF

Little is known about the perception of artificial spatial hearing by hearing-impaired subjects. The purpose of this study was to investigate how listeners with hearing disorders perceived the effect of a spatialization feature designed for wireless microphone systems. Forty listeners took part in the experiments.

View Article and Find Full Text PDF
Article Synopsis
  • Recent research has improved our understanding of dyserythropoiesis, particularly through studies on β-thalassemia, which may lead to new treatments for anemia.
  • The review highlights significant proteins like GATA-1, HSP70, TGF-β1, GDF-11, and erythroferrone that are involved in this condition, offering potential therapeutic targets.
  • Notably, drugs like sotatercept and lustatercept, which inhibit GDF-11, show promise for treating conditions related to ineffective erythropoiesis, including β-thalassemia and myelodysplastic syndromes.
View Article and Find Full Text PDF

Receptor-interacting protein kinase 3 (RIPK3) mediates necroptosis, a form of programmed cell death that promotes inflammation in various pathological conditions, suggesting that it might be a privileged pharmacological target. However, its function in glucose homeostasis and obesity has been unknown. Here we show that RIPK3 is over expressed in the white adipose tissue (WAT) of obese mice fed with a choline-deficient high-fat diet.

View Article and Find Full Text PDF

Purpose Of Review: The review provides an overview of recent data regarding the molecular players in β-thalassemia dyserythropoiesis and the corresponding therapeutic implications.

Recent Findings: β-thalassemia dyserythropoiesis is characterized by four steps: expansion of erythroid progenitors, accelerated erythroid differentiation until the polychromatophilic stage, maturation arrest, and apoptosis at the polychromatophilic stage. Excess α-globin chains are the primary culprit in the disease, but the link between this excess and ineffective erythropoiesis has only recently been established.

View Article and Find Full Text PDF

Mycoplasma faucium has recently been associated with brain abscesses and seems to originate from the mouth. We evaluated its prevalence by quantitative real-time PCR (qPCR) in the oropharynxes of 644 subjects and found that 25% harbored M. faucium, probably constituting the gateway for entrance of the bacteria into cerebral abscesses.

View Article and Find Full Text PDF

The transcription factor NF-κB plays a key role in numerous physiological processes such as inflammation, immunity, cell proliferation or control of cell death. Its activation is tightly controlled by a kinase complex, IκB kinase (IKK), composed of three core proteins: IKK1/IKKα, IKK2/IKKβ and NEMO/IKKγ. The first two are structurally related kinases whereas the third one is a regulatory subunit exhibiting affinity for upstream activators modified by polyubiquitin chains.

View Article and Find Full Text PDF

β-Thalassaemia major (β-TM) is an inherited haemoglobinopathy caused by a quantitative defect in the synthesis of β-globin chains of haemoglobin, leading to the accumulation of free α-globin chains that form toxic aggregates. Despite extensive knowledge of the molecular defects causing β-TM, little is known of the mechanisms responsible for the ineffective erythropoiesis observed in the condition, which is characterized by accelerated erythroid differentiation, maturation arrest and apoptosis at the polychromatophilic stage. We have previously demonstrated that normal human erythroid maturation requires a transient activation of caspase-3 at the later stages of maturation.

View Article and Find Full Text PDF