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4-methylumbelliferyl iduronide
fluorogenic substrate for (alpha-L)-iduronidase
Also Known As:
4-MUBI; 4-methylumbelliferyl-alpha-L-iduronide; alpha-L-Idopyranosiduronic acid, 4-methyl-2-oxo-2H-1-benzopyran-7-yl
Networked:
4
relevant articles (
0
outcomes,
0
trials/studies)
Bio-Agent Context: Research Results
Heterocyclic Compounds: 198
1-Ring Heterocyclic Compounds
Pyrans: 130
Benzopyrans: 195
Coumarins: 826
Umbelliferones: 3
Hymecromone: 123
4-methylumbelliferyl iduronide: 4
Fused-Ring Heterocyclic Compounds
2-Ring Heterocyclic Compounds
Benzopyrans: 195
Coumarins: 826
Umbelliferones: 3
Hymecromone: 123
4-methylumbelliferyl iduronide: 4
Related Diseases
1.
Mucopolysaccharidosis I (Hurler Syndrome)
04/01/1980 - "
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.
"
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.
"
01/01/1991 - "
Activity of alpha-L-iduronidase was studied in leukocytes of healthy persons, of patients with Hurler disease and of heterozygous carriers of the disease where 4-methylumbelliferyl-alpha-L-iduronide and 4-trifluoromethylumbelliferyl-alpha-L-iduronide were used as substrates.
"
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.
"
01/01/1991 - "
It was shown that 4-trifluoromethylumbelliferyl-alpha-L-iduronide, along with the commonly used 4-methylumbelliferyl-alpha-L-iduronide, can serve as a specific substrate for alpha-L-iduronidase and is therefore suitable for demonstrating the enzyme deficiency in patients with Hurler disease, as well as the decrease of enzyme activity in heterozygous disease carriers.
"
Related Drugs and Biologics
1.
Iduronidase (alpha-L-Iduronidase)
2.
4-trifluoromethylumbelliferyl iduronide
3.
Enzymes
4.
phenyl-alpha-L-iduronide