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Properties of α-l-iduronidase in cultured skin fibroblasts from α-l-iduronidase-deficient patients

✍ Scribed by S. Fujibayashi; R. Minami; Y. Ishikawa; K. Wagatsuma; T. Nakao; S. Tsugawa

Publisher: Springer
Year: 1984
Tongue: English
Weight: 324 KB
Volume: 65
Category: Article
ISSN: 0340-6717
DOI: 10.1007/bf00286515

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✦ Synopsis

On DEAE cellulose column chromatography, alpha-L-iduronidase in cultured skin fibroblasts was resolved into two distinct components, forms A and B. They had similar Km values for 4-methylumbelliferyl-alpha-L-iduronide, but differed in pH optima and thermal stability. Form B was more heat-stable than form A. Residual alpha-L-iduronidase activity in Hurler fibroblasts was heat-stable, while that in Scheie fibroblasts was heat-labile, and moreover, that in Hurler-Scheie compound fibroblasts lay intermediate between Hurler and Scheie syndromes. These findings demonstrated that Hurler syndrome, Scheie syndrome and Hurler-Scheie compound were enzymatically distinguishable.

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