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phenyl-alpha-L-iduronide

subsrtate for alpha-L-iduronidase

Also Known As:

PLIDN; alpha-L-Idopyranosiduronic acid, phenyl

Networked: 2 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Related Diseases

1.

Mucopolysaccharidosis I (Hurler Syndrome)

10/08/1981 - "The activities of alpha-L-iduronidase (EC 3.2.1.76) were measured, by a microassay with phenyl-alpha-L-iduronide as the substrate, in cultured fibroblasts from patients with the Hurler syndrome (MPS IH) or the Scheie syndrome (MPS IS) and from heterozygotes.l The iduronidase activities in the cells from 44 MPS IH and 7 MPS IS patients were all strongly reduced and distinct from the activities in heterozygotes and normal controls. "
01/01/1980 - "Comparison of the substrate 4-methylumbelliferyl-alpha-l-iduronide with phenyl-alpha-L-iduronide for the diagnosis of Hurler's disease in cultured cells."
01/01/1980 - "4-Methylumbelliferyl-alpha-l-iduronide provided a more sensitive method than phenyl-alpha-l-iduronide for the estimation of alpha-l-iduronidase in cultured cells and could be used to diagnose Hurler's disease. "

Related Drugs and Biologics

1.	Iduronidase (alpha-L-Iduronidase)
2.	4-methylumbelliferyl iduronide