- An insight into the biochemistry of inborn errors of metabolism for a clinical neurologist

The oligosaccharides in urine are produced by the incomplete breakdown of the carbohydrate side chains of complex glycoproteins. Analysis of urine oligosaccharides is an inexpensive, although not very specific, screening test for glycoproteinoses. The most widely used method is thin layer chromatography of diluted urine. Increased amounts of free sialic acid are excreted in the case of sialic acid storage disorders and can be determined in urine by spectrophotometry.

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