Géographie d'une filière émergente : le bois-énergie

International audienc

Table-ronde de conclusion

International audienc

Evolution de la chalarose du frêne en Wallonie

Depuis quelques années, la maladie du frêne, la chalarose, cause des dégâts catastrophiques dans les frênaies européennes, au point de poser la question de l'avenir du frêne. Avec cet article, l'Observatoire wallon de la santé des forêts fait le point sur la situation en Wallonie et propose des voies de gestion des peuplements affectés par cette maladie

Emotional Speech Comprehension in Deaf Children with Cochlear Implant

International audienceAbstract We examined the understanding of emotional speech by deaf children with cochlear implant (CI). Thirty deaf children with CI and 60 typically developing controls (matched on chronological age or hearing age) performed a computerized task featuring emotional prosody, either embedded in a discrepant context or without any context at all. Across the task conditions, the deaf participants with CI scored lower on the prosody-bases responses than their peers matched on chronological age or hearing age. Additionally, we analyzed the effect of age on determining correct prosody-based responses and we found that hearing age was a predictor of the accuracy of prosody-based responses. We discuss these findings with respect to delay in prosody and intermodal processing. Future research should aim to specify the nature of the cognitive processes that would be required to process prosody

Emotional Speech Comprehension in Deaf Children with Cochlear Implant

We examined the understanding of emotional speech by deaf children with cochlear implant (CI). Thirty deaf children with CI and 60 typically developing controls (matched on chronological age or hearing age) performed a computerized task featuring emotional prosody, either embedded in a discrepant context or without any context at all. Across the task conditions, the deaf participants with CI scored lower on the prosody-bases responses than their peers matched on chronological age or hearing age. Additionally, we analyzed the effect of age on determining correct prosody-based responses and we found that hearing age was a predictor of the accuracy of prosody-based responses. We discuss these findings with respect to delay in prosody and intermodal processing. Future research should aim to specify the nature of the cognitive processes that would be required to process prosody

Reactive Astrocytes Overexpress TSPO and Are Detected by TSPO Positron Emission Tomography Imaging

International audienceAstrocytes and microglia become reactive under most brain pathological conditions, making this neuroinflammation process a surrogate marker of neuronal dysfunction. Neuroinflammation is associated with increased levels of translocator protein 18 kDa (TSPO) and binding sites for TSPO ligands. Positron emission tomography (PET) imaging of TSPO is thus commonly used to monitor neuroinflammation in preclinical and clinical studies. It is widely considered that TSPO PET signal reveals reactive microglia, although a few studies suggested a potential contribution of reactive astrocytes. Because astrocytes and microglia play very different roles, it is crucial to determine whether reactive astro-cytes can also overexpress TSPO and yield to a detectable TSPO PET signal in vivo. We used a model of selective astrocyte activation through lentiviral gene transfer of the cytokine ciliary neurotrophic factor (CNTF) into the rat striatum, in the absence of neurodegeneration. CNTF induced an extensive activation of astrocytes, which overexpressed GFAP and become hypertrophic, whereas microglia displayed minimal increaseinreactivemarkers.TwoTSPOradioligands,[ 18 F]DPA-714[N,N-diethyl-2-(2-(4-(2-[ 18 F]fluoroethoxy)phenyl)-5,7-dimethylpyrazolo[1, 5-a]pyrimidin-3-yl)acetamide] The volume of radioligand binding matched the GFAP immunopositive volume. TSPO mRNA levels were significantly increased, and TSPO protein was overexpressed by CNTF-activated astrocytes. We show that reactive astrocytes overexpress TSPO, yielding to a significant and selective binding of TSPO radioligands. Therefore, caution must be used when interpreting TSPO PET imaging in animals or patients because reactive astrocytes can contribute to the signal in addition to reactive microglia

Phenotypic spectrum and genomics of undiagnosed arthrogryposis multiplex congenita

Arthrogryposis multiplex congenita (AMC) is characterised by congenital joint contractures in two or more body areas. AMC exhibits wide phenotypic and genetic heterogeneity. Our goals were to improve the genetic diagnosis rates of AMC, to evaluate the added value of whole exome sequencing (WES) compared with targeted exome sequencing (TES) and to identify new genes in 315 unrelated undiagnosed AMC families

Gain-of-function and loss-of-function variants in GRIA3 lead to distinct neurodevelopmental phenotypes

International audienceAbstract AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid) receptors (AMPARs) mediate fast excitatory neurotransmission in the brain. AMPARs form by homo- or heteromeric assembly of subunits encoded by the GRIA1-GRIA4 genes, of which only GRIA3 is X-chromosomal. Increasing numbers of GRIA3 missense variants are reported in patients with neurodevelopmental disorders (NDD), but only a few have been examined functionally. Here, we evaluated the impact on AMPAR function of one frameshift and 43 rare missense GRIA3 variants identified in patients with NDD by electrophysiological assays. Thirty-one variants alter receptor function and show loss-of-function (LoF) or gain-of-function (GoF) properties, whereas 13 appeared neutral. We collected detailed clinical data from 25 patients (from 23 families) harbouring 17 of these variants. All patients had global developmental impairment, mostly moderate (9/25) or severe (12/25). Twelve patients had seizures, including focal motor (6/12), unknown onset motor (4/12), focal impaired awareness (1/12), (atypical) absence (2/12), myoclonic (5/12), and generalized tonic-clonic (1/12) or atonic (1/12) seizures. The epilepsy syndrome was classified as developmental and epileptic encephalopathy in eight patients, developmental encephalopathy without seizures in 13 patients, and intellectual disability with epilepsy in four patients. Limb muscular hypotonia was reported in 13/25, and hypertonia in 10/25. Movement disorders were reported in 14/25, with hyperekplexia or non-epileptic erratic myoclonus being the most prevalent feature (8/25). Correlating receptor functional phenotype with clinical features revealed clinical features for GRIA3-associated NDDs and distinct NDD phenotypes for LoF and GoF variants. GoF variants were associated with more severe outcomes: patients were younger at the time of seizure onset (median age one month), hypertonic, and more often had movement disorders, including hyperekplexia. Patients with LoF variants were older at the time of seizure onset (median age 16 months), hypotonic, and had sleeping disturbances. LoF and GoF variants were disease-causing in both sexes but affected males often carried de novo or hemizygous LoF variants inherited from healthy mothers, whereas all but one affected females had de novo heterozygous GoF variants