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

Excess acidic mucopolysaccharides in urine can be detected and quantified by measuring the change in color of metachromatic dyes such as Alcian blue, dimethylmethylene blue or toluidine blue. Although qualitative spot tests, such as the Berry spot test, are rapid and inexpensive, they produce many false-positive and false-negative results. Uni- or bidirectional electrophoresis or thin layer chromatography of urinary mucopolysaccharides provides information that is important for the classification of the disorders by indicating the relative proportions of specific mucopolysaccharides.

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