Fluorometric measurement of alpha-L-iduronidase activity using 4-methylumbelliferyl-alpha-L-iduronide.

Abstract

Using 4-methylumbelliferyl-alpha-L-iduronide as a substrate, alpha-L-iduronidase activity was measured in leukocytes and in lymphoblastoid cells obtained from patients with alpha-L-iduronidase deficiency and from obligate heterozygotes for this disease. There was complete discrimination between alpha-L-iduronide in leukocytes and in lymphoblastoid cells from the patients and controls. However, overlap was observed between values of the activity in the obligate heterozygotes and those in the controls. 4-Methylumbelliferyl-alpha-L-iduronidase activity because of greater sensitivity, easier assay procedure and shorter incubation period.

• Authors: R Minami, Y Watanabe, T Kudoh, K Oyanagi, T Nakao
• Journal: The Tohoku journal of experimental medicine (Tohoku J Exp Med) Vol. 130 Issue 4 Pg. 381-4 (Apr 1980) ISSN: 0040-8727 [Print] Japan
• PMID: 6781103 (Publication Type: Journal Article)

Chemical References

• Umbelliferones
• Iduronic Acid
• Hymecromone
• 4-methylumbelliferyl iduronide
• Glycoside Hydrolases
• Iduronidase

Topics

• Female
• Fluorometry
• Glycoside Hydrolases (blood)
• Humans
• Hymecromone (analogs & derivatives)
• Iduronic Acid (analogs & derivatives)
• Iduronidase (blood, deficiency)
• Male
• Mucopolysaccharidoses (enzymology)
• Mucopolysaccharidosis I (enzymology)
• Umbelliferones