3 results match your criteria: "Centre National de la Recherche Scinetifique[Affiliation]"

Topical Sodium Thiosulfate: A Treatment for Calcifications in Hyperphosphatemic Familial Tumoral Calcinosis?

J Clin Endocrinol Metab

July 2016

Pharmacie (J.J., V.R.), Inserm, Université de Limoges, Centre Hospitalo Universitaire de Limoges, Unité Mixte de Recherche Scientifique 1094, Institut d'Epidémiologie Neurologique et de Neurologie tropicale, Centre National de la Recherche Scinetifique Fédération de Recherche 3503 Génomique, Environnement, Immunité, Santé et Thérapie Pédiatrie (C.B., A.Lie., H.M., V.G.), Comité de l'HME pour la Recherche Clinique Centre Hospitalier Universitaire; and Centre d'Investigation Clinique (E.P.), Centre Hospitalier Universitaire, 87000 Limoges, France; Assistance Publique-Hôpitaux de Paris (M.C.), Hôpital Européen Georges Pompidou, Département de Physiologie; Université Paris Descartes, Faculté de Médecine; and Institut Necker Enfants-Malades, Inserm Unité 1151, Centre National de la Recherche Scientifique Unité Mixte de Recherche 8253, 75000 Paris, France; Pédiatrie (T.-A.T.), Centre Hospitalier Universitaire de Nîmes; Inserm Unité 1183, Faculté de Médecine Montpellier-Nîmes, 30000 Nîmes France; Assistance Publique-Hôpitaux de Paris (A.Lin.), Centre de Référence des Maladies Rare du Métabolisme Phosphocalcique et Plateforme d'Expertise Paris Sud Maladies Rares; and Inserm Unité 1169, Le Kremlin Bicêtre 94270, France; Génétique Médicale (K.B.), Hôpital Raymond Poincaré, 92380 Garches, France; and Centre National de la Recherche Scinetifique Unité Mixte de Recherche 7276 (V.G.), Université de Limoges, 87000 Limoges, France.

Context: Hyperphosphatemic familial tumoral calcinosis (HFTC) and hyperphosphatemia hyperostosis syndrome (HHS) are rare diseases characterized by hyperphosphatemia and ectopic calcifications or recurrent episodes of diaphysitis. In the setting of metabolic or inflammatory diseases, recent data suggest that systemic administration of sodium thiosulfate (STS) could be effective in the treatment of ectopic calcifications but may also be poorly tolerated (digestive symptoms, metabolic acidosis). Our group developed a topical formulation of STS to treat ectopic calcifications locally, therefore limiting patient exposure to the drug and its adverse effects.

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The benzodiazepine Midazolam mitigates the breathing defects of Mecp2-deficient mice.

Respir Physiol Neurobiol

June 2011

Unité Mixte de Recherche (UMR) 6231, Centre National de la Recherche Scinetifique, Université de la Méditerranée, Université Paul Cézanne, Faculté Saint Jérôme (Service 362), Marseille, France.

Rett syndrome is a severe neurodevelopmental disease caused by mutations of the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2) that induce complex, disabling symptoms, including breathing symptoms. Males of Mecp2-deficient mice (Mecp2(-/y)) normally breathe at birth but develop first altered breathing regulations, thereafter erratic breathing with severe apnoeas, aggravating until respiratory distress and premature death. Mecp2(-/y) mice also develop early GABA deficits.

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The interaction of factor Va with phospholipid monolayers was studied using alternating current polarography. For these studies a hanging mercury drop electrode is positioned in contact with the monolayer at the air-monolayer interface. Factor Va introduced into the aqueous subphase beneath the monolayer caused alterations in the capacitance of the circuit.

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