SPOT4 Management Centre

In the context of the CNES SPOT4 program CISI is particularly responsible for the development of the SPOT4 Management Centre, part of the SPOT4 ground control system located at CNES Toulouse (France) designed to provide simultaneous control over two satellites. The main operational activities are timed to synchronize with satellite visibilities (ten usable passes per day). The automatic capability of this system is achieved through agenda services (sequence of operations as defined and planned by operator). Therefore, the SPOT4 Management Centre offers limited, efficient and secure human interventions for supervision and decision making. This paper emphasizes the main system characteristics as degree of automation, level of dependability and system parameterization

Genetic Variant in HK1 Is Associated With a Proanemic State and A1C but Not Other Glycemic Control–Related Traits

OBJECTIVE A1C is widely considered the gold standard for monitoring effective blood glucose levels. Recently, a genome-wide association study reported an association between A1C and rs7072268 within HK1 (encoding hexokinase 1), which catalyzes the first step of glycolysis. HK1 deficiency in erythrocytes (red blood cells [RBCs]) causes severe nonspherocytic hemolytic anemia in both humans and mice. RESEARCH DESIGN AND METHODS The contribution of rs7072268 to A1C and the RBC-related traits was assessed in 6,953 nondiabetic European participants. We additionally analyzed the association with hematologic traits in 5,229 nondiabetic European individuals (in whom A1C was not measured) and 1,924 diabetic patients. Glucose control–related markers other than A1C were analyzed in 18,694 nondiabetic European individuals. A type 2 diabetes case-control study included 7,447 French diabetic patients. RESULTS Our study confirms a strong association between the rs7072268–T allele and increased A1C (β = 0.029%; P = 2.22 × 10−7). Surprisingly, despite adequate study power, rs7072268 showed no association with any other markers of glucose control (fasting- and 2-h post-OGTT–related parameters, n = 18,694). In contrast, rs7072268–T allele decreases hemoglobin levels (n = 13,416; β = −0.054 g/dl; P = 3.74 × 10−6) and hematocrit (n = 11,492; β = −0.13%; P = 2.26 × 10−4), suggesting a proanemic effect. The T allele also increases risk for anemia (836 cases; odds ratio 1.13; P = 0.018). CONCLUSIONS HK1 variation, although strongly associated with A1C, does not seem to be involved in blood glucose control. Since HK1 rs7072268 is associated with reduced hemoglobin levels and favors anemia, we propose that HK1 may influence A1C levels through its anemic effect or its effect on glucose metabolism in RBCs. These findings may have implications for type 2 diabetes diagnosis and clinical management because anemia is a frequent complication of the diabetes state

GNAS-activating mutations define a rare subgroup of inflammatory liver tumors characterized by STAT3 activation.

International audienceBACKGROUND & AIMS: Mosaic G-protein alpha-subunit (GNAS)-activating mutations are responsible for the McCune-Albright (MCA) syndrome. This oncogene that activates the adenylate cyclase is also mutated in various tumor types leading to the accumulation of cyclic-AMP. Identification of a hepatocellular adenoma (HCA) in two MCA patients led us to search for GNAS activation in benign and malignant hepatocellular carcinogenesis. METHODS: GNAS mutations were screened by sequencing 164 HCA, 245 hepatocellular carcinoma (HCC), and 17 fibrolamellar carcinomas. Tumors were characterized by quantitative RT-PCR, gene mutation screening and pathological reviewing. The consequences of wild type and mutant GNAS expression were analyzed in hepatocellular cell lines. RESULTS: A somatic GNAS-activating mutation was identified in 5 benign tumors and in 2 HCC. In benign tumors, GNAS mutations were exclusive from HNF1A, CTNNB1, and IL6ST mutations whereas one HCC demonstrated both CTNNB1 and GNAS mutations. Quantitative RT-PCR showed an activation of the IL-6 and interferon pathways in GNAS-mutated tumor tissues. Accordingly, pathological reviewing identified in GNAS-mutated tumors an inflammatory phenotype characterized by fibrosis and STAT3 activation. We further demonstrated in HCC cell lines that GNAS mutant expression induced inflammatory response and STAT3 activation. CONCLUSIONS: We identified for the first time the association between two rare diseases, MCA syndrome and HCA occurrence, but also that somatic GNAS-activating mutations in sporadic benign and malignant liver tumors are characterized by an inflammatory phenotype. These results showed a cross-talk between cyclic-AMP and JAK/STAT pathways in liver tumors and they reinforce the role of STAT3 activation in liver tumorigenesis

Prêtres et pasteurs

La fracture confessionnelle qui touche l’Occident chrétien au xvie siècle révolutionne durablement la place des clergés dans les sociétés et leur emprise sur les fidèles. Le principe originel du luthéranisme, puis des réformes de type suisse, du sacerdoce universel a été largement relativisé par la mise en place progressive d’un clergé protestant, mais il a ouvert une brèche que l’on peut suivre à l’époque des controverses et qui continue de fixer une différence fondamentale entre les deux camps. Pourtant, des influences réciproques et des contacts sont observables dans des zones de coexistence confessionnelle : les espaces de frontières peuvent faire figure d’observatoires privilégiés pour comprendre les influences réciproques entre clergés, jusque dans leur façon d’interpréter et de considérer le ministère pastoral et de forger leurs identités professionnelles et sociales. La formation des clergés, leur organisation, leur action, les oppositions auxquelles ils doivent faire face, leurs interactions, les points communs, voire les sociabilités de leurs membres sont ici étudiés à travers des pratiques très diverses (prédication, conférences et controverses, sociabilités érudites, missions, encadrement institutionnel, pratiques liturgiques, répression des déviances, ou encore définition de mémoires concurrentes). Ces études de cas nous plongent dans le processus de définition d’identités cléricales qui se constituent certes souvent contre l’autre, mais aussi, plus subtilement, en fonction de l’autre et sur des fondements communs

Prêtres et pasteurs

La fracture confessionnelle qui touche l’Occident chrétien au xvie siècle révolutionne durablement la place des clergés dans les sociétés et leur emprise sur les fidèles. Le principe originel du luthéranisme, puis des réformes de type suisse, du sacerdoce universel a été largement relativisé par la mise en place progressive d’un clergé protestant, mais il a ouvert une brèche que l’on peut suivre à l’époque des controverses et qui continue de fixer une différence fondamentale entre les deux camps. Pourtant, des influences réciproques et des contacts sont observables dans des zones de coexistence confessionnelle : les espaces de frontières peuvent faire figure d’observatoires privilégiés pour comprendre les influences réciproques entre clergés, jusque dans leur façon d’interpréter et de considérer le ministère pastoral et de forger leurs identités professionnelles et sociales. La formation des clergés, leur organisation, leur action, les oppositions auxquelles ils doivent faire face, leurs interactions, les points communs, voire les sociabilités de leurs membres sont ici étudiés à travers des pratiques très diverses (prédication, conférences et controverses, sociabilités érudites, missions, encadrement institutionnel, pratiques liturgiques, répression des déviances, ou encore définition de mémoires concurrentes). Ces études de cas nous plongent dans le processus de définition d’identités cléricales qui se constituent certes souvent contre l’autre, mais aussi, plus subtilement, en fonction de l’autre et sur des fondements communs

Clinical heterogeneity and phenotype/genotype findings in 5 families with &ITGYG1&IT deficiency

International audienceObjective: To describe the variability of muscle symptoms in patients carrying mutations in the GYG1 gene, encoding glycogenin-1, an enzyme involved in the biosynthesis of glycogen, and to discuss genotype-phenotype relations.Methods: We describe 9 patients from 5 families in whom muscle biopsies showed vacuoles with an abnormal accumulation of glycogen in muscle fibers, partially alpha-amylase resistant suggesting polyglucosan bodies. The patients had either progressive early-onset limb-girdle weakness or late-onset distal or scapuloperoneal muscle affection as shown by muscle imaging. No clear definite cardiac disease was found. Histologic and protein analysis investigations were performed on muscleResults: Genetic analyses by direct or exome sequencing of the GYG1 gene revealed 6 different GYG1 mutations. Four of the mutations were novel. They were compound heterozygous in 3 families and homozygous in 2. Protein analysis revealed either the absence of glycogenin-1 or reduced glycogenin-1 expression with impaired glucosylation.Conclusions: Our report extends the genetic and clinical spectrum of glycogenin-1-related myopathies to include scapuloperoneal and distal affection with glycogen accumulation

Clinical heterogeneity and phenotype/genotype findings in 5 families with &ITGYG1&IT deficiency

International audienceObjective: To describe the variability of muscle symptoms in patients carrying mutations in the GYG1 gene, encoding glycogenin-1, an enzyme involved in the biosynthesis of glycogen, and to discuss genotype-phenotype relations.Methods: We describe 9 patients from 5 families in whom muscle biopsies showed vacuoles with an abnormal accumulation of glycogen in muscle fibers, partially alpha-amylase resistant suggesting polyglucosan bodies. The patients had either progressive early-onset limb-girdle weakness or late-onset distal or scapuloperoneal muscle affection as shown by muscle imaging. No clear definite cardiac disease was found. Histologic and protein analysis investigations were performed on muscleResults: Genetic analyses by direct or exome sequencing of the GYG1 gene revealed 6 different GYG1 mutations. Four of the mutations were novel. They were compound heterozygous in 3 families and homozygous in 2. Protein analysis revealed either the absence of glycogenin-1 or reduced glycogenin-1 expression with impaired glucosylation.Conclusions: Our report extends the genetic and clinical spectrum of glycogenin-1-related myopathies to include scapuloperoneal and distal affection with glycogen accumulation

Clinical heterogeneity and phenotype/genotype findings in 5 families with<i> GYG1 </i> deficiency

International audienceObjective: To describe the variability of muscle symptoms in patients carrying mutations in the GYG1 gene, encoding glycogenin-1, an enzyme involved in the biosynthesis of glycogen, and to discuss genotype-phenotype relations.& para;& para;Methods: We describe 9 patients from 5 families in whom muscle biopsies showed vacuoles with an abnormal accumulation of glycogen in muscle fibers, partially alpha-amylase resistant suggesting polyglucosan bodies. The patients had either progressive early-onset limb-girdle weakness or late-onset distal or scapuloperoneal muscle affection as shown by muscle imaging. No clear definite cardiac disease was found. Histologic and protein analysis investigations were performed on muscle.& para;& para;Results: Genetic analyses by direct or exome sequencing of the GYG1 gene revealed 6 different GYG1 mutations. Four of the mutations were novel. They were compound heterozygous in 3 families and homozygous in 2. Protein analysis revealed either the absence of glycogenin-1 or reduced glycogenin-1 expression with impaired glucosylation.& para;& para;Conclusions: Our report extends the genetic and clinical spectrum of glycogenin-1-related myopathies to include scapuloperoneal and distal affection with glycogen accumulation