Progressive Ataxia and Palatal Tremor: Think about POLG Mutations

Background: Progressive ataxia and palatal tremor (PAPT) can be observed in both acquired brainstem or cerebellar lesions and genetic disorders. Phenomenology shown: PAPT due to mutation in POLG, the gene encoding the mitochondrial DNA polymerase. Educational value: POLG mutation should be considered in patients with PAPT, particularly when additional clues such as a sensory neuronopathy or an ophthalmoplegia are present

pKa tuning in quadrupolar-type two-photon ratiometric fluorescent membrane probes

International audienceTwo bolaamphiphilic quadrupoles built from a fluorene core conjugated with azine endgroups were designed and successfully used to stain GUV membranes. Their quadrupolar character induces both a shift of the pKa values close to the physiological pH and large two-photon absorption responses (i.e. over 1000 GM for acidic forms). As such they hold promise as ratiometric two-photon pH probes for monitoring slight variations of pH near cell membranes using two-photon excitation in the NIR regio

Nonlinear optical properties of meso-Tetra(fluorenyl)porphyrins peripherally functionalized with one to four ruthenium alkynyl substituents

The synthesis of a series of four porphyrin derivatives based on a meso-tetrafluorenylporphyrin core functionalized with one to four trans-chlorobis(dppe)ruthenium alkynyl units (dppe = 1,2-bis(diphenylphosphino)ethane) at the periphery, together with cyclic voltammetry (CV) and UV–Vis absorption and emission spectroscopy studies, are reported. In these multipolar assemblies, the organoruthenium endgroups are potential electron-donors and the central porphyrin core is a potential electron-acceptor. The third-order nonlinear optical (NLO) responses have been assessed by Z-scan, revealing that these extended π-networks incorporating polarizable organometallic units behave as nonlinear absorbers in the near-IR range. The role of the peripheral transition metal centers on the third-order NLO properties is discussed

Influence of the synthetic method on the properties of two-photon-sensitive mesoporous organosilica nanoparticles

International audienceHerein we report the modulation of the properties of mesoporous silica nanoparticles (NPs) via various synthetic approaches. Three types of elaborations were compared, one in aqueous media at 25 °C, and the other two at 80 °C in water or in a water–ethanol mixture. For all these methods, an alkoxysilylated two-photon photosensitizer (2PS) was co-condensed with tetraethylorthosilicate (TEOS) in the presence of cetyltrimethylammonium bromide (CTAB), leading to five two-photon-sensitive mesoporous silica (M2PS) NPs. The M2PS NP porous structure could be tuned from radial to worm-like and MCM-41 types of organization. Besides, the 2PS precursor spatial dispersion was found to be highly dependent on both the 2PS initial concentration and the elaboration process. As a result, two-photon properties were modulated by the choice of the synthesis, the best results being found in aqueous media at 25 or 80 °C. Finally, the M2PS NPs were used for in vitro two-photon imaging of cancer cells

Letter Ruling 99-1: Electronic Retailers

The synthesis and characterization of a set of redox-active iron and ruthenium alkynyl complexes of general formula [[M]­Cl_{(1–<i>p</i>)}{CCC₆H_5–<i>m</i>(CCFlu)_<i>m</i>}_(1+<i>p</i>)]­[PF₆]_<i>n</i> are reported (<i>n</i> = 0–1; <i>m</i> = 1–2; [M] = [Fe­(η⁵-C₅Me₅)­(κ²-dppe)] and <i>p</i> = 1 or [M] = [<i>trans</i>-Ru­(κ²-dppe)₂] and <i>p</i> = 0–1). The linear and third-order nonlinear optical properties of these new organometallic complexes featuring phenylalkynyl ligands functionalized by 2-fluorenyl (Flu) groups were studied in their stable redox states. Their first electronic transitions are assigned with the help of DFT calculations. We show here that these compounds possess significant third-order NLO responses in the near-IR range for molecules of their size. In particular, the remarkably large 2PA activities of the new Ru­(II) compounds in the 600–800 nm range (<i>Z</i>-scan) make them attractive nonlinear chromophores. Structure–property studies emphasize the importance of para- versus meta-connection of the 2-fluorenylethynyl units on the phenylalkynyl core and reveal that upon progressing from mono- to bis-alkynyl complexes a further increase of the 2PA cross section can be obtained while maintaining linear transparency in the visible range

Linear and Third-Order Nonlinear Optical Properties of Fe(n5-C5Me5)(k2-dppe)- and trans-Ru(k2-dppe)2-Alkynyl Complexes Containing 2-Fluorenyl End Groups

The synthesis and characterization of a set of redox-active iron and ruthenium alkynyl complexes of general formula [[M]Cl(1–p){C≡CC6H5–m(C≡CFlu)m}(1+p)][PF6]n are reported (n = 0–1; m = 1–2; [M] = [Fe(η5-C5Me5)(κ2-dppe)] and p = 1 or [M] = [trans-Ru(κ2-dppe)2] and p = 0–1). The linear and third-order nonlinear optical properties of these new organometallic complexes featuring phenylalkynyl ligands functionalized by 2-fluorenyl (Flu) groups were studied in their stable redox states. Their first electronic transitions are assigned with the help of DFT calculations. We show here that these compounds possess significant third-order NLO responses in the near-IR range for molecules of their size. In particular, the remarkably large 2PA activities of the new Ru(II) compounds in the 600–800 nm range (Z-scan) make them attractive nonlinear chromophores. Structure–property studies emphasize the importance of para- versus meta-connection of the 2-fluorenylethynyl units on the phenylalkynyl core and reveal that upon progressing from mono- to bis-alkynyl complexes a further increase of the 2PA cross section can be obtained while maintaining linear transparency in the visible range

Etude en torsion d'arcs orthodontiques à base de titane-molybdène

NANCY1-SCD Pharmacie-Odontologie (543952101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Paroxysmal Exercise-induced Dyskinesias Caused by GLUT1 Deficiency Syndrome

Background: Glucose transporter type 1 deficiency syndrome is due to de novo mutations in the SLC2A1 gene encoding the glucose transporter type 1. Phenomenology Shown: Paroxysmal motor manifestations induced by exercise or fasting may be the main manifestations of glucose transporter type 1 deficiency syndrome. Educational Value: Proper identification of the paroxysmal events and early diagnosis is important since the disease is potentially treatable.</p

LES ORIGINES DE LA DISTINCTION ENTRE POSITIF ET NORMATIF EN ECONOMIE (The Origin of the Positive-normative Distinction in Economics)

Economists are accustomed to distinguishing between a positive and a normative component of their work, a distinction that is peculiar to their field, having no exact counterpart in the other social sciences. The distinction has substantially changed over time, and the different ways of understanding it today are reflective of its history. Our objective is to trace the origins and initial forms of the distinction, from the English classical political economy of the first half of the 19th century to the emergence of welfare economics in the first half of the 20th century. This sequential account will also serve to identify the main representative positions along with the arguments used to support them, and it thus prepares the ground for a discussion that will be less historical and more strictly conceptual.Les économistes ont coutume de distinguer entre une composante positive et une composante normative de leurs travaux, ce qui est une singularité de leur discipline, car cette distinction n'a pas de répondant exact dans les autres sciences sociales. Elle a fortement évolué au cours du temps et les différentes manières de la concevoir aujourd'hui en reflètent l'histoire. On se propose ici d'en retracer les origines et les premières formes, de l'économie politique classique anglaise de la première moitié du XIXe siècle jusqu'à l'apparition de l'économie du bien-être dans la première moitié du XXe siècle. Ce parcours séquentiel vise aussi à identifier les positions les plus représentatives et les arguments invoqués pour les soutenir, en préparant ainsi une discussion qui serait moins historique et plus strictement conceptuelle

Interannual variability of net community production and air-sea CO₂ flux in a naturally iron fertilized region of the Southern Ocean (Kerguelen Plateau)

International audienc