Title : Immunoquantification and enzyme kinetics of alpha-L-iduronidase in cultured fibroblasts from normal controls and mucopolysaccharidosis type I patients.

Pub. Date : 1992 Apr

PMID : 1550122






1 Functional Relationships(s)
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1 alpha-L-Iduronidase activity is deficient in mucopolysaccharidosis type I (MPS I; Hurler syndrome, Scheie syndrome) patients and results in the disruption of the sequential degradation of the glycosaminoglycans dermatan sulfate and heparan sulfate. Glycosaminoglycans alpha-L-iduronidase Homo sapiens