4-Methylumbelliferyl-α-L-iduronide 2-sulfate (sodium)
4-Methylumbelliferyl-α-L-iduronide 2-sulfate (4-MU-α-idoa 2-sulfate) sodium is a fluorogenic substrate of iduronate-2-sulfatase (I2S). 4-Methylumbelliferyl-α-L-iduronide 2-sulfate sodium can be hydrolyzed by lysosomal enzyme α-l-iduronidase (IDUA) and release the fluorophore 4-Methylumbelliferone. 4-Methylumbelliferyl-α-L-iduronide 2-sulfate sodium can be used to detect Hurler syndrome[1][2].
Product Specifications
CAS Number
2181774-46-7
Product Name Alternative
4-MU-α-idoa 2-sulfate (sodium)
UNSPSC
12352005
Target
Fluorescent Dye
Related Pathways
Others
Field of Research
Metabolic Disease
Smiles
O=S(O[C@H]([C@H]([C@@H]([C@@H](O1)C(O[Na])=O)O)O)[C@@H]1OC2=CC=C(C(C)=CC(O3)=O)C3=C2)(O[Na])=O
Molecular Formula
C16H14Na2O12S
Molecular Weight
476.32
References & Citations
[1]Azadeh M, et al. A Rapid Two-Step Iduronate-2-Sulfatatse Enzymatic Activity Assay for MPSII Pharmacokinetic Assessment. JIMD Rep. 2018;38:89-95. |[2]Gusarova VD, et al. Characterization of a HIR-Fab-IDS, Novel Iduronate 2-Sulfatase Fusion Protein for the Treatment of Neuropathic Mucopolysaccharidosis Type II (Hunter Syndrome) . BioDrugs. 2023 May;37 (3) :375-395.
Shipping Conditions
Room temperature
Scientific Category
Reference compound2
Clinical Information
No Development Reported
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