Publisher Summary This chapter provides an overview of the primary structure of glycoprotein glycans. There exists a certain unity of structure among glycans. All are derived by the substitution of common, core oligosaccharides ( inv fractions) by variable oligosaccharide structures ( var fractions), which are the basis of their specificity. The var fractions present a limited number of the types of structure. In the case of N -glycosylproteins, in which the glycan-protein bond is asparaginyl-2-acetamido-2-deoxyglucose, the glycans are of three fundamental types: the oligomannosidic, the N -acetyllactosaminic, and the mixed, oligomannosidic- N -acetyl-lactosaminic. The determination of the structures of oligosaccharides from the urines of glycoproteinoses, diseases characterized by a deficit in lysosomal enzymes catabolizing the glycans of glycoprotein, allows reconstruction of the different stages of glycoprotein catabolism, and presentation of the hypothesis that the catabolism of N -acetyl-lactosainine glycans starts by the action of endo-2-acetamido-2-deoxy-β- D -glucosidases in liberating oligosaccharides that are then degraded by exoglycosidases. This chapter elaborates the primary structure and metabolism of the N -linked glycans. Development and improvement of procedures for the study of the primary structure of glycans is also discussed.

Primary structure of glycoprotein glycans: basis for the molecular biology of glycoproteins.

Pompe’s disease is a neuromuscular disorder caused by deficiency of lysosomal acid α–glucosidase. Recombinant human α– glucosidase is under evaluation as therapeutic drug. In light of this development we studied the natural course of cases not fitting the definition of classic infantile Pompe’s disease. Our review of 109 reports including 225 cases shows a continuous spectrum of phenotypes. The onset of symptoms ranged from 0 to 71 years. Based on the available literature, no criteria to delineate clinical sub–types could be established.

The natural course of non-classic Pompe's disease; a review of 225 published cases.

Post-translational modification of proteins.

https://www.jbc.org/content/276/43/40338.full.pdf

Modification of epidermal growth factor-like repeats with O-fucose. Molecular cloning and expression of a novel GDP-fucose protein O-fucosyltransferase.

Glycosyltransferases and their use in assessing oligosaccharide structure and structure-function relationships.

A new type of carbohydrate-protein linkage in a glycopeptide from normal human urine.

. A glucose-containing tetrasaccharide (13 mg/24 h) was isolated from the urine of a ten year old boy with the “childhood form” of glycogen storage disease type II (Pompe's disease). The structure, established by sugar analysis, methylation analysis, optical rotation and enzymatic degradation was found to be α-D-Glc p (lr6)-α-D-Glc p (l r 4).α-D-Glc p (1r4)-D-Glc. The same compound was also isolated in small amounts (1 mg/24 h) from normal urine. Larger glucose-containing oligosaccharides, not detected in normal urine were also present in the urine of the Pompe case.

Increased Excretion of a Glucose‐Containing Tetrasaccharide in the Urine of a Patient with Glycogen Storage Disease Type II (Pompe's Disease)

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A new type of carbohydrate-protein linkage in a glycopeptide from normal human urine.

Increased Excretion of a Glucose‐Containing Tetrasaccharide in the Urine of a Patient with Glycogen Storage Disease Type II (Pompe's Disease)