Diagnostic du déficit immunitaire primitif du au défaut d’expression des molécules HLA de classe II au Maroc

L’expression membranaire des molécules du complexe majeur d’histocompatibilité de classe II (CMH-II) joue un rôle crucial dans les phénomènes de présentation des antigènes aux lymphocytes T CD4+ et de déclenchement de la réponse immune. Les déficits immunitaires dus au défaut d’expression de ces molécules constituent un ensemble hétérogène d’affections héréditaires rares de transmission autosomique récessive. Ce désordre est dû à la présence d’une anomalie génétique de l’un des 4 gènes CIITA, RFXANK, RFX5 et RFXAP codants pour les protéines impliquées dans la régulation de la transcription des gènes CMH II. La majorité des cas rapportés dans la littérature sont d’origine maghrébine. Ce déficit est caractérisé cliniquement par de nombreuses infections notamment digestives et pulmonaires qui apparaissent tôt dans la vie. C’est une affection encore méconnue dans notre pays par les cliniciens. Ce travail résume la stratégie de diagnostic du déficit des molécules CMH II suivie par l’Unité d’Immunologie Clinique du CHU à Casablanca qui a introduit pour la première fois au Maroc, le diagnostic moléculaire en collaboration avec l’Institut Pasteur du Maroc de Casablanca

Significance of Cuscutain, a cysteine protease from Cuscuta reflexa, in host-parasite interactions

<p>Abstract</p> <p>Background</p> <p>Plant infestation with parasitic weeds like <it>Cuscuta reflexa </it>induces morphological as well as biochemical changes in the host and the parasite. These modifications could be caused by a change in protein or gene activity. Using a comparative macroarray approach <it>Cuscuta </it>genes specifically upregulated at the host attachment site were identified.</p> <p>Results</p> <p>One of the infestation specific <it>Cuscuta </it>genes encodes a cysteine protease. The protein and its intrinsic inhibitory peptide were heterologously expressed, purified and biochemically characterized. The haustoria specific enzyme was named cuscutain in accordance with similar proteins from other plants, e.g. papaya. The role of cuscutain and its inhibitor during the host parasite interaction was studied by external application of an inhibitor suspension, which induced a significant reduction of successful infection events.</p> <p>Conclusions</p> <p>The study provides new information about molecular events during the parasitic plant - host interaction. Inhibition of cuscutain cysteine proteinase could provide means for antagonizing parasitic plants.</p

Effects of a balanced translocation between chromosomes 1 and 11 disrupting the DISC1 locus on white matter integrity

Objective Individuals carrying rare, but biologically informative genetic variants provide a unique opportunity to model major mental illness and inform understanding of disease mechanisms. The rarity of such variations means that their study involves small group numbers, however they are amongst the strongest known genetic risk factors for major mental illness and are likely to have large neural effects. DISC1 (Disrupted in Schizophrenia 1) is a gene containing one such risk variant, identified in a single Scottish family through its disruption by a balanced translocation of chromosomes 1 and 11; t(1;11) (q42.1;q14.3). Method Within the original pedigree, we examined the effects of the t(1;11) translocation on white matter integrity, measured by fractional anisotropy (FA). This included family members with (n = 7) and without (n = 13) the translocation, along with a clinical control sample of patients with psychosis (n = 34), and a group of healthy controls (n = 33). Results We report decreased white matter integrity in five clusters in the genu of the corpus callosum, the right inferior fronto-occipital fasciculus, acoustic radiation and fornix. Analysis of the mixed psychosis group also demonstrated decreased white matter integrity in the above regions. FA values within the corpus callosum correlated significantly with positive psychotic symptom severity. Conclusions We demonstrate that the t(1;11) translocation is associated with reduced white matter integrity in frontal commissural and association fibre tracts. These findings overlap with those shown in affected patients with psychosis and in DISC1 animal models and highlight the value of rare but biologically informative mutations in modeling psychosis

Techno-economic performance evaluation of direct steam generation solar tower plants with thermal energy storage systems based on high-temperature concrete and encapsulated phase change materials

Nowadays, direct steam generation concentrated solar tower plants suffer from the absence of a cost-effective thermal energy storage integration. In this study, the prefeasibility of a combined sensible and latent thermal energy storage configuration has been performed from thermodynamic and economic standpoints as a potential storage option. The main advantage of such concept with respect to only sensible or only latent choices is related to the possibility to minimize the thermal losses during system charge and discharge processes by reducing the temperature and pressure drops occurring all along the heat transfer process. Thermodynamic models, heat transfer models, plant integration and control strategies for both a pressurized tank filled with sphere-encapsulated salts and high temperature concrete storage blocks were developed within KTH in-house tool DYESOPT for power plant performance modeling. Once implemented, cross-validated and integrated the new storage model in an existing DYESOPT power plant layout, a sensitivity analysis with regards of storage, solar field and power block sizes was performed to determine the potential impact of integrating the proposed concept. Even for a storage cost figure of 50 USD/kWh, it was found that the integration of the proposed storage configuration can enhance the performance of the power plants by augmenting its availability and reducing its levelized cost of electricity. As expected, it was also found that the benefits are greater for the cases of smaller power block sizes. Specifically, for a power block of 80 MWe a reduction in levelized electricity costs of 8% was estimated together with an increase in capacity factor by 30%, whereas for a power block of 126 MWe the benefits found were a 1.5% cost reduction and 16% availability increase