Oligosaccharides, Urinary

Table 4.2.1 lists the disorders of glycoprotein degradation that are detected by urinary oligosaccharide screening. It is beyond the scope of this article to give clinical descriptions of all of the disorders listed therefore the reader is referred to the references given in Table 4.2.1. 

Figure 4.2.2 depicts the urinary oligosaccharide patterns of sialidosis (a-neur-aminidase deficiency), GM1 gangliosidosis and mucopolysaccharidosis type IVB (/3-galaclosidase deficiency) compared to a normal urine and standards of raffinose, lactose and glucose. In patients with sialidosis, a densely staining band close to the... 

Fig. 4.2.2 TLC of urinary oligosaccharides. Left to right standards of raf-finose (lower), lactose (middle) and glucose (upper), sialidosis, GM1 gangliosidosis, mucopolysaccharidosis IVB and a control...

Klein A, Lebreton A, Lemoine J, Perini J-M, Roussel P, Michalski J-C (1998) Identification of urinary oligosaccharides by matrix-assisted laser desorption ionisation time-of-flight mass spectrometry. Clin Chem 44 2422-2428... 

Kuczynski TW, Kendzierski KS, Sewell AC (1993) Urinary oligosaccharides in pregnant or lactating women a pitfall in screening. Clin Chem 39 2346-2347... 

Rudloff S, Pohlentz G, Diekmann L, Egge H, Kunz C (1996) Urinary excretion of lactose and oligosaccharides in preterm infants fed human milk or infant formula. Acta Paediatr... 

Sewell AC (1979) An improved thin-layer chromatographic method for urinary oligosaccharide screening. Clin Chim Acta 92 411-414... 

Sewell AC (1986) Urinary oligosaccharide screening detects type VI glycogen storage disease. Clin Chem 32 392... 

Tsai MY, Marshall JG (1979) Screening for urinary oligosaccharides and simple sugars by thin-layer chromatography. Med Lab Sci 36 85-90... 

Variations in the quantities and structures of urinary oligosaccharides may offer a diagnostic test for pathological conditions. They reflect a deficiency of a-L-fucosidase activity, leading to excretion of more of the fucosylated saccharides than in normal subjects. 

This variation in results is, in part, a reflection of the variation in the methods that have been used for isolating urinary glycopeptides and peptido-oligosaccharides, and, from this, it would appear that there is, as yet, no generally accepted, methodical sequence for this... 

Carbohydrate and Peptide Composition of Urinary Glycopeptides and Peptido-oligosaccharides... 

Durand, G and Seta, N., Protein glycosylation and diseases Blood and urinary oligosaccharides as markers for diagnosis and therapeutic monitoring, Clin. Chem., 46, 795, 2000. 

An increased urinary excretion of the D-glucosyl tetrasaccharide (44) has been detected in patients with Duchenne muscular dystrophy.This oligosaccharide was originally reported in the urine of a patient with glycogen storage disease type II (Pompe s disease). 

Work on the search for a biochemical basis of six different types of sialidosis have centred on the use of two radiolabelled oligosaccharides prepared from human urinary oligosaccharides (12) and (13). ... 

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