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4-Methylumbelliferyl α-L-iduronide (free acid) (CAS 66966-09-4)

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Alternate Names:
4MU alpha-L-iduronide (free acid), 4-Methylumbelliferyl iduronide, 4-methyl-2-oxo-2h-chromen-7-yl-α-l-idopyranosiduronic acid
Application:
4-Methylumbelliferyl α-L-iduronide (free acid) is a fluorogenic substrate for α-L-iduronidase.
CAS Number:
66966-09-4
Molecular Weight:
352.29
Molecular Formula:
C16H16O9
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
* Refer to Certificate of Analysis for lot specific data.

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4-Methylumbelliferyl alpha-L-iduronide (free acid) is used in biochemical research as a fluorogenic substrate for assaying the activity of the enzyme alpha-L-iduronidase. This enzyme activity is for the degradation of glycosaminoglycans, specifically dermatan sulfate and heparan sulfate. In the context of research applications, 4-Methylumbelliferyl alpha-L-iduronide (free acid) is employed to measure enzyme kinetics as well as to screen for deficiencies in alpha-L-iduronidase activity, which is pertinent in the study of mucopolysaccharidosis type I (MPS I). Upon cleavage by the enzyme, the compound releases a fluorescent 4-methylumbelliferone moiety, which can be easily detected and quantified. This feature facilitates the monitoring of enzyme activity in various assay conditions and the investigation of factors that influence the activity of alpha-L-iduronidase. Moreover, the use of this substrate aids in the exploration of enzyme function in normal and pathological states, contributing to a deeper understanding of metabolic disorders involving lysosomal storage.


4-Methylumbelliferyl α-L-iduronide (free acid) (CAS 66966-09-4) References

  1. Fluorometric measurement of urinary alpha-L-iduronidase activity.  |  Isemura, M., et al. 1978. J Biochem. 84: 627-32. PMID: 102641
  2. A fluorometric assay using 4-methylumbelliferyl alpha-L-iduronide for the estimation of alpha-L-iduronidase activity and the detection of Hurler and Scheie syndromes.  |  Hopwood, JJ., et al. 1979. Clin Chim Acta. 92: 257-65. PMID: 114339
  3. Retrovirally mediated correction of bone marrow-derived mesenchymal stem cells from patients with mucopolysaccharidosis type I.  |  Baxter, MA., et al. 2002. Blood. 99: 1857-9. PMID: 11861306
  4. [Use of 4-methylumbelliferryl-alpha-L-iduronide and 4-trifluoromethylumbelliferryl-alpha-L-iduronide for detecting alpha-L-iduronidase deficiencies in human tissue and for rapid prenatal diagnosis of Hurler disease].  |  Tsvetkova, IV., et al. 1991. Vopr Med Khim. 37: 74-7. PMID: 1907053
  5. Use of 4-trifluoromethylumbelliferyl-alpha-L-iduronide as a new substrate for detection of alpha-L-iduronidase deficiency in human tissues and for rapid prenatal diagnosis of Hurler disease.  |  Tsvetkova, IV., et al. 1991. J Inherit Metab Dis. 14: 134-9. PMID: 1909400
  6. Multiplex newborn screening for Pompe, Fabry, Hunter, Gaucher, and Hurler diseases using a digital microfluidic platform.  |  Sista, RS., et al. 2013. Clin Chim Acta. 424: 12-8. PMID: 23660237
  7. Toward reducing immunogenicity of enzyme replacement therapy: altering the specificity of human β-glucuronidase to compensate for α-iduronidase deficiency.  |  Chuang, HY., et al. 2015. Protein Eng Des Sel. 28: 519-29. PMID: 26319005
  8. Direct demonstration of binding of a lysosomal enzyme, alpha-L-iduronidase, to receptors on cultured fibroblasts.  |  Rome, LH., et al. 1979. Proc Natl Acad Sci U S A. 76: 2331-4. PMID: 287076
  9. Human alpha-L-iduronidase. 2. Catalytic properties.  |  Clements, PR., et al. 1985. Eur J Biochem. 152: 29-34. PMID: 4043083
  10. Human alpha-L-iduronidase. II. Comparative biochemical and immunologic properties of the purified low and high uptake forms.  |  Schuchman, EH., et al. 1984. Enzyme. 31: 166-75. PMID: 6734578
  11. Fluorometric measurement of alpha-L-iduronidase activity using 4-methylumbelliferyl-alpha-L-iduronide.  |  Minami, R., et al. 1980. Tohoku J Exp Med. 130: 381-4. PMID: 6781103
  12. Comparison of the substrate 4-methylumbelliferyl-alpha-l-iduronide with phenyl-alpha-L-iduronide for the diagnosis of Hurler's disease in cultured cells.  |  Butterworth, J. and Broadhead, DM. 1980. J Inherit Metab Dis. 2: 71-4. PMID: 6796763
  13. Studies on the alpha-L-iduronidase activity of beta-glucuronidase preparations from bovine liver, rat liver, and rat preputial gland.  |  Kosaka, H., et al. 1980. J Biochem. 88: 69-75. PMID: 7410341

Ordering Information

Product NameCatalog #UNITPriceQtyFAVORITES

4-Methylumbelliferyl α-L-iduronide (free acid), 2 mg

sc-262101
2 mg
$434.00

4-Methylumbelliferyl α-L-iduronide (free acid), 10 mg

sc-262101A
10 mg
$2128.00

4-Methylumbelliferyl α-L-iduronide (free acid), 50 mg

sc-262101B
50 mg
$10407.00

4-Methylumbelliferyl α-L-iduronide (free acid), 100 mg

sc-262101C
100 mg
$20808.00

Hello, What is the Extinction coefficient of 4-Methylumbelliferyl α-L-iduronide (free acid)? Thank you

Asked by: Samin Ardalani
Thank you for your question. The extinction coefficient of this compound was not determined.
Answered by: Tech Support Europe
Date published: 2023-12-04

hi, how is the purity and thermal stability of this drug

Asked by: SYang
Thank you for your question. Purity is typically >97%. To prevent deterioration, the product should be stored in tightly closed containers, in a freezer or cold room at -20 degrees centigrade. The product may deteriorate slightly if exposed to light and humidity for prolonged periods.
Answered by: Technical Support
Date published: 2017-09-29

Do you have any information on long term storage in solution?

Asked by: SCchem16
Thank you for your question. This chemical, 4-Methylumbelliferyl α-L-iduronide (free acid): sc-262101, should be stored long term desiccated at -20ºC in tightly closed containers. The product may deteriorate if exposed to light and humidity for prolonged periods of time. We recommend solutions be used the same day they are made.
Answered by: Chemical Support 2
Date published: 2017-02-27

How accurate is the 2mg weight?

Asked by: SCchem16
Thank you for your question. This chemical, 4-Methylumbelliferyl α-L-iduronide (free acid) (CAS 66966-09-4), is measured out between 2.1mg and 2.4mg per vial.
Answered by: Chemical Support 2
Date published: 2017-02-27

How is this material best solublized as a stock solution. Thanks.

Asked by: kendrickgoss
Thank you for your inquiry! sc-262101 (4-Methylumbelliferyl α-L-iduronide (free acid)) is soluble under the following conditions: 1% solution in 1% acetic acid/methanol 70:30 v/v. The following article that may contain information pertinent for using this product as well: http://www.ncbi.nlm.nih.gov/pubmed/114339
Answered by: Tech Service
Date published: 2016-09-26
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Rated 5 out of 5 by from Various publications cite 4Various publications cite 4-Methylumbelliferyl -L-iduronide as a fluorogenic substrate for -L-iduronidase. -SCBT Publication Review
Date published: 2015-05-28
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4-Methylumbelliferyl α-L-iduronide (free acid) is rated 5.0 out of 5 by 1.
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