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Human airway mucins represent a very broad family of polydisperse high molecular mass glycoproteins, which are part of the airway innate immunity. Apomucins, which correspond to their peptide part, are encoded by at least 6 different mucin genes (MUC1, MUC2, MUC4, MUC5B, MUC5AC and MUC7). The expression of some of these genes (at least MUC2 and MUC5AC) is(More)
The structure of sialylated carbohydrate units of bronchial mucins obtained from cystic fibrosis patients was investigated by 500-MHz 1H NMR spectroscopy in conjunction with sugar analysis. After subjecting the mucins to alkaline borohydride degradation, sialylated oligosaccharide-alditols were isolated by anion-exchange chromatography and fractionated by(More)
Human respiratory mucins consist of a family of glycoproteins with different peptides in which glycosylation, the major post-translational phenomenon, is responsible for about 70 to 80% of the weight of these molecules. This glycosylation generates a remarkable diversity of O-glycosidically linked carbohydrate chains, which are expressed as several hundreds(More)
Sialic acids can be acetylated at the 4, 7, 8 and/or 9 position. Biological roles of these substitutions have been missed until recently because of their low abundance and lability to conventional purification methods. The recent advances in the analysis of sialic acids have allowed to demonstrate that O-acetylation has a selective but widespread(More)
Glycoproteinoses belong to the lysosomal storage disorders group. The common feature of these diseases is the deficiency of a lysosomal protein that is part of glycan catabolism. Most of the lysosomal enzymes involved in the hydrolysis of glycoprotein carbohydrate chains are exo-glycosidases, which stepwise remove terminal monosaccharides. Thus, the(More)
PURPOSE N-glycosylation modifications in human serum glycoproteins have been described in hepatic cirrhosis. To identify the glycoproteins carrying these modifications and to determine their influences in the modification of the total serum N-glycome (TSNG) in cirrhotic patients, we have performed the glycosylation analysis of immunoglobulins, transferrin,(More)
Sialic acids are a family of 9-carbon carboxylated sugars, where different substitutions of the backbone define over 30 members. Biological roles of these substitutions have been missed until recently because of their low abundance and lability to conventional isolation/purification methods. This new approach characterizes sialic acids using electrospray(More)
Appearance of a hyposialylated transferrin fraction in the plasma during chronic alcohol exposure is a well-known phenomenon, and it represents the best available marker of chronic alcohol consumption. The mechanisms of its appearance are still not well understood and are extremely complex, involving biosynthesis and catabolism alterations, although the(More)
The human total serum N-glycome (TSNG) is the representation of the serum glycoproteins N-glycosylation. The study of N-glycosylation in clinical biochemistry laboratories is emerging as a new assay for diagnosis of congenital and acquired diseases. This review describes the N-glycosylation of serum glycoproteins and its contribution to the serum N-glycome,(More)