Abstract Congenital disorders of glycosylation (CDG) are metabolic hereditary diseases caused by defects in the synthesis of glycoconjugates. CDG have been described in sugar-nucleotide biosynthesis and transporter, glycosyltransferases, vesicular transport, as well as in lipid biosynthesis and glycosylphosphatidylinositol anchors. PMM2-CDG is caused by mutations in the phosphomannomutase-2 (PMM2) gene and shows autosomal recessive inheritance. It affects all organs and tissues, ranging from severe psychomotor retardation to moderate intellectual disability. Alterations in the primary haemostatic system have been reported in these patients and they can lead to severe bleeding or excessive thrombosis with subsequent vascular insufficiency. Despite of being the most common CDG, platelet glycosylation and sialylation defects in PMM2-CDG patients remain incompletely characterized. In this study, we applied a lectin-based flow cytometry approach to report the first characterization of the highly glycosylated platelet membrane glycan profile in a PMM2-CDG patient. In the PMM2-CDG patient’s platelet samples, a decreased binding of SNA lectin, indicative of reduced terminal α-2-6 sialic acid content, and an increased binding of PNA lectin, suggesting desialylation of β-1-N-acetylgalactosamine residues, were observed. Reduced expression of terminal sialic acids in platelet membrane glycoproteins may contribute to the increased risk of hemorrhage reported in these patients by promoting platelet clearance and thrombocytopenia.

In this work LaNiO3 oxide was prepared by a self-combustion method using citric acid. The electrodes were obtained by coating a nickel foam support with the oxide suspension. Optical microscopy and cyclic voltammetry were used on the electrodes characterization. The evaluation of the electrodes electrocatalytic activity, towards the oxygen evolution reaction in alkaline medium, was performed by means of steady state measurements. The reaction follows a first order kinetics, with respect to OH- concentration, with Tafel slopes close to 40 mV, for low overpotentials. Based on the apparent and real current densities it was possible to conclude that the increase on the electrode activity, when compared with the published data, is mostly related to geometric factors. This fact has been associated with the high electrode/electrolyte contact area provided by the foam nickel substrate. Synergetic effects between the Ni foam and the perovskite oxide cannot be discarded.

The electrochemical oxidation of four different azo dyes, C.I. Acid Orange 7 (AO7), C. I. Direct Red 254 (DR254), C. I. Direct Red 80 (DR80) and Yellow Gold Sandolan (YGS), was performed using as anode material an oxide with a perovskite like structure, BaPb0.9Sb0.1O3-δ. Bulk electrolysis was studied using Na2SO4 as electrolyte, at a current density of 5 mA cm-2. UV-Visible absorbance measurements, Chemical Oxygen Demand (COD), Total Organic Carbon (TOC) and High Performance Liquid Chromatography (HPLC) analysis were performed, in order to follow the colour and COD removals and the mineralization index. The obtained results show an almost complete colour removal for the solutions containing AO7, DR254 or DR80, after 24 h essay. For these dyes, after 96 h electrodegradation experiment, COD removals between 30 and 70 % and TOC removals ranging from 15 to 40 % were obtained. However, for the experimental conditions used, the electrodegradation of YGS was not possible.