Open source business strategies in the domain of embedded systems

El tema de este estudio es el aumento de la comprensión teórica y empírica de la estrategia de negocio de código abierto en el dominio de sistemas embebidos por investigar modelos de negocios de código abierto, retos, recursos y capacidades operativas y dinámicas.

Novel mutation in the tyrosine kinase domain of FGFR2 in a patient with Pfeiffer syndrome.

Mutations in the fibroblast growth factor receptor 2 (FGFR2) cause a variety of craniosynostosis syndromes. The mutational spectrum tends to be narrow with the majority of mutations occurring in either exon IIIa or IIIc or in the intronic sequence preceding exon IIIc. Mutations outside of this hotspot are uncommon and the few identified mutations have demonstrated wide clinical variability, making it difficult to establish a clear-cut genotype-phenotype correlation. To better delineate the clinical picture associated with these unusual mutations, we describe a severely affected patient with Pfeiffer syndrome and a missense mutation in the tyrosine kinase (TK) domain of FGFR2.

Orientation Center Newsletter, Winter 2004

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Orientation Center Newsletter, Summer 2003

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Orientation Center Newsletter, Summer 2004

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Orientation Center Newsletter, Summer 2005

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Orientation Center Newsletter, Summer 2006

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Construire sa vie (Life designing): Un paradigme pour l'orientation au 21e siècle

La nouvelle organisation sociale du travail du début du 21e siècle pose une série de questions et lance de nombreux défis aux spécialistes de l'accompagnement en orientation professionnelle. Compte tenu de la mondialisation du conseil en orientation, c'est dans un cadre international que nous avons décidé d'aborder ces questions, puis de formuler des réponses potentiellement innovantes. Cette approche permet d'éviter les difficultés que soulève la création de modèles et de méthodes dans un pays donné en tentant ensuite de les exporter dans d'autres, où ils doivent être adaptés pour être utilisés. Cet article présente le premier résultat tangible de cette collaboration : un modèle et des méthodes d'accompagnement des personnes. Le modèle « Construire sa vie », destiné à des interventions d'accompagnement en orientation se fonde sur cinq présupposés concernant les personnes et leur vie professionnelle : des possibilités liées aux contextes, des processus dynamiques, une progression non linéaire, des perspectives multiples et des configurations individuelles. En partant de ces présupposés, nous avons bâti un modèle en contexte, se réclamant de l'épistémologie du constructionnisme social, reconnaissant en particulier que les connaissances et l'identité d'un individu sont le produit d'interactions sociales et que le sens est co-construit, via la médiation du discours. Le cadre général « construire sa vie » s'appuie sur les théories de la construction de soi (Guichard, 2005) et de la construction des parcours professionnels (Savickas, 2005), qui décrivent les conduites d'orientation et leurs développements. Ce cadre concerne toute la vie, il est holiste, tient compte des contextes et est préventif.

The death domain-containing protein Unc5CL is a novel MyD88-independent activator of the pro-inflammatory IRAK signaling cascade.

The family of death domain (DD)-containing proteins are involved in many cellular processes, including apoptosis, inflammation and development. One of these molecules, the adapter protein MyD88, is a key factor in innate and adaptive immunity that integrates signals from the Toll-like receptor/interleukin (IL)-1 receptor (TLR/IL-1R) superfamily by providing an activation platform for IL-1R-associated kinases (IRAKs). Here we show that the DD-containing protein Unc5CL (also known as ZUD) is involved in a novel MyD88-independent mode of IRAK signaling that culminates in the activation of the transcription factor nuclear factor kappa B (NF-κB) and c-Jun N-terminal kinase. Unc5CL required IRAK1, IRAK4 and TNF receptor-associated factor 6 but not MyD88 for its ability to activate these pathways. Interestingly, the protein is constitutively autoproteolytically processed, and is anchored by its N-terminus specifically to the apical face of mucosal epithelial cells. Transcriptional profiling identified mainly chemokines, including IL-8, CXCL1 and CCL20 as Unc5CL target genes. Its prominent expression in mucosal tissues, as well as its ability to induce a pro-inflammatory program in cells, suggests that Unc5CL is a factor in epithelial inflammation and immunity as well as a candidate gene involved in mucosal diseases such as inflammatory bowel disease.

L'orientation du patient souffrant d'un syndrome douloureux abdominal aigu a domicile [Hospitalisation criteria for the acute abdominal pain patient seen at home or in an ambulatory setting].

Dealing at patient's home with an acute abdominal pain may be particularly challenging for the primary care physician. In such a clinical situation, the part of laboratory and radiological investigations is increasing in the diagnostic process. The decision to keep the patient at home based on a clinical evaluation alone may represent a great medical responsibility for the physician. Emergency departments (ED) are of course in charge of investigating such patients with a wide panel of investigation techniques. But these structures are chronically overcrowded resulting frequently in long and difficult periods of waiting. Based on a literature review, a description of useful clinical symptoms and signs is summarized and should help the decision process for the orientation of the patient.

Pathogenic Vibrio activate NLRP3 inflammasome via cytotoxins and TLR/nucleotide-binding oligomerization domain-mediated NF-kappa B signaling.

Vibrio vulnificus and Vibrio cholerae are Gram-negative pathogens that cause serious infectious disease in humans. The beta form of pro-IL-1 is thought to be involved in inflammatory responses and disease development during infection with these pathogens, but the mechanism of beta form of pro-IL-1 production remains poorly defined. In this study, we demonstrate that infection of mouse macrophages with two pathogenic Vibrio triggers the activation of caspase-1 via the NLRP3 inflammasome. Activation of the NLRP3 inflammasome was mediated by hemolysins and multifunctional repeat-in-toxins produced by the pathogenic bacteria. NLRP3 activation in response to V. vulnificus infection required NF-kappaB activation, which was mediated via TLR signaling. V. cholerae-induced NLRP3 activation also required NF-kappaB activation but was independent of TLR stimulation. Studies with purified V. cholerae hemolysin revealed that toxin-stimulated NLRP3 activation was induced by TLR and nucleotide-binding oligomerization domain 1/2 ligand-mediated NF-kappaB activation. Our results identify the NLRP3 inflammasome as a sensor of Vibrio infections through the action of bacterial cytotoxins and differential activation of innate signaling pathways acting upstream of NF-kappaB.

Orientation Center Newsletter, August, 2007, Vol. 27, no. 1

The Orientation Center newsletter is produced three times a year, and includes articles written by students, staff, and former students. It also contains news about what is happening to other students who have been in the Center.

Recurrent dominant mutations affecting two adjacent residues in the motor domain of the monomeric kinesin KIF22 result in skeletal dysplasia and joint laxity.

Spondyloepimetaphyseal dysplasia with joint laxity, leptodactylic type (lepto-SEMDJL, aka SEMDJL, Hall type), is an autosomal dominant skeletal disorder that, in spite of being relatively common among skeletal dysplasias, has eluded molecular elucidation so far. We used whole-exome sequencing of five unrelated individuals with lepto-SEMDJL to identify mutations in KIF22 as the cause of this skeletal condition. Missense mutations affecting one of two adjacent amino acids in the motor domain of KIF22 were present in 20 familial cases from eight families and in 12 other sporadic cases. The skeletal and connective tissue phenotype produced by these specific mutations point to functions of KIF22 beyond those previously ascribed functions involving chromosome segregation. Although we have found Kif22 to be strongly upregulated at the growth plate, the precise pathogenetic mechanisms remain to be elucidated.