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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Status of newborn screening and follow up investigations for Mucopolysaccharidoses I and II in Taiwan

Fig. 2

a The method utilizes 4-methylumbelliferyl-α-L-iduronide substrate that is hydrolyzed by IDUA into a highly fluorescent product, 4-methylumbelliferone (4MU). The rate of fluorescence increase is directly proportional to enzyme activity. b The enzymatic liberation of the fluorochrome from 4MU-α-L-iduronide-2sulfate requires the sequential action of IDS and α-iduronidase

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172