480 research outputs found

    Pituitary adenylate cyclase-activating polypeptide deficiency associated with increased platelet count and aggregability in nephrotic syndrome

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    The pituitary adenylate cyclase-activating polypeptide (PACAP) was recently identified as an inhibitor of megakaryopoiesis and platelet aggregability.sponsorship: This work was supported by grants from the Fund for Scientific Research, Flanders (F. W. O. Vlaanderen, B. Eneman, C. van Geet, and E. Levtchenko) and by a Bayer and Norbert Heimburger Chair (C. van Geet). S. S. Staes is thanked for her technical assistance. (Fund for Scientific Research, Flanders, Bayer and Norbert Heimburger Chair)status: Publishe

    Distal renal tubular acidosis: ERKNet/ESPN clinical practice points

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    Trepiccione, F., Walsh, S.B., Ariceta, G., Boyer, O., Emma, F., Camilla, R., Ferraro, P.M., Haffner, D., Konrad, M., Levtchenko, E., Lopez-Garcia, S.C., Santos, F., Stabouli, S., Szczepanska, M., Tasic, V., Topaloglu, R., Vargas-Poussou, R., Wlodkowski, T., Bockenhauer, D

    Parathyroid hormone and phosphate homeostasis in patients with Bartter and Gitelman syndrome: an international cross-sectional study

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    European Reference Network for Rare Kidney Diseases (ERKNet) - European Union; Dutch Kidney Foundation [19OI06]Verploegen MFA, Vargas-Poussou R, Walsh SB, Alpay H, Amouzegar A, Ariceta G, Atmis B, Bacchetta J, Bárány P, Baron S, Bayrakci US, Belge H, Besouw M, Blanchard A, Bökenkamp A, Boyer O, Burgmaier K, Calò LA, Decramer S, Devuyst O, van Dyck M, Ferraro PM, Fila M, Francisco T, Ghiggeri GM, Gondra L, Guarino S, Hooman N, Hoorn EJ, Houillier P, Kamperis K, Kari JA, Konrad M, Levtchenko E, Lucchetti L, Lugani F, Marzuillo P, Mohidin B, Neuhaus TJ, Osman A, Papizh S, Perelló M, Rookmaaker MB, Conti VS, Santos F, Sawaf G, Serdaroglu E, Szczepanska M, Taroni F, Topaloglu R, Trepiccione F, Vidal E, Wan ER, Weber L, Yildirim ZY, Yüksel S, Zlatanova G, Bockenhauer D, Emma F, Nijenhuis

    Evidence of non-Dzyaloshinskii–Moriya ferromagnetism in epitaxial BiFeO3 films

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    X-ray diffraction analysis and high-resolution electron microscopy of BiFeO3 films prepared by dc magnetron sputtering on single-crystal LaAlO3 (001) substrates reveal that the films have a highly c-oriented orthorhombic crystalline structure. The magnetic properties of the BiFeO3 films are typical of ensembles of interacting superparamagnetic clusters, rather than Dzyaloshinskii-Moriya weak ferromagnets. The appearance of extrinsic nanoscale superparamagnetic clusters is explained by an oxygen deficiency in certain regions of the film, where ferromagnetic ordering can be realized through a double-exchange Zener mechanism.Applied Science

    Molecular and functional characterization of urine-derived podocytes from patients with Alport syndrome

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    Alport syndrome (AS) is a genetic disorder involving mutations in the genes encoding collagen IV α3, α4 or α5 chains, resulting in the impairment of glomerular basement membrane. Podocytes are responsible for production and correct assembly of collagen IV isoforms; however, data on the phenotypic characteristics of human AS podocytes and their functional alterations are currently limited. The evident loss of viable podocytes into the urine of patients with active glomerular disease enables their isolation in a non-invasive way. Here we isolated, immortalized, and subcloned podocytes from the urine of three different AS patients for molecular and functional characterization. AS podocytes expressed a typical podocyte signature and showed a collagen IV profile reflecting each patient's mutation. Furthermore, RNA-sequencing analysis revealed 348 genes differentially expressed in AS podocytes compared with control podocytes. Gene Ontology analysis underlined the enrichment in genes involved in cell motility, adhesion, survival, and angiogenesis. In parallel, AS podocytes displayed reduced motility. Finally, a functional permeability assay, using a podocyte–glomerular endothelial cell co-culture system, was established and AS podocyte co-cultures showed a significantly higher permeability of albumin compared to control podocyte co-cultures, in both static and dynamic conditions under continuous perfusion. In conclusion, our data provide a molecular characterization of immortalized AS podocytes, highlighting alterations in several biological processes related to extracellular matrix remodelling. Moreover, we have established an in vitro model to reproduce the altered podocyte permeability observed in patients with AS. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland

    Heavy-quark pseudoscalar formation in two-photon collisions at LEP

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    A search for the pseudoscalar mesons eta /sub c/ and eta /sub b/, produced by two-photon interactions is performed using the L3 data collected at centre-of-mass energies from 183 GeV to 209 GeV, for a total integrated luminosity of 610 pb/sup -1/. (9 refs)
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