M9766
4-Methylumbelliferyl α-D-glucopyranoside
α-glucosidase substrate, fluorogenic, ≥99% (TLC), powder
Synonym(s):
4-Methylumbelliferyl α-D-glucoside
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About This Item
Empirical Formula (Hill Notation):
C16H18O8
CAS Number:
Molecular Weight:
338.31
NACRES:
NA.32
PubChem Substance ID:
UNSPSC Code:
12352204
EC Number:
241-794-0
MDL number:
Beilstein/REAXYS Number:
1690776
Product Name
4-Methylumbelliferyl α-D-glucopyranoside, α-glucosidase substrate
Quality Level
assay
≥99% (TLC)
form
powder
solubility
pyridine: 50 mg/mL, clear, colorless to faintly yellow
storage temp.
−20°C
SMILES string
CC1=CC(=O)Oc2cc(O[C@H]3O[C@H](CO)[C@@H](O)[C@H](O)[C@H]3O)ccc12
InChI
1S/C16H18O8/c1-7-4-12(18)23-10-5-8(2-3-9(7)10)22-16-15(21)14(20)13(19)11(6-17)24-16/h2-5,11,13-17,19-21H,6H2,1H3/t11-,13-,14+,15-,16+/m1/s1
InChI key
YUDPTGPSBJVHCN-JZYAIQKZSA-N
Application
4-Methylumbelliferyl ǥ-D-glucopyranoside has been used to assay acid alpha-glucosidase (GAA) activity in tissue homogenates.
Biochem/physiol Actions
4-Methylumbelliferyl ǥ-D-glucopyranoside serves as a fluorogenic substrate for the ǥ-glucosidase enzyme. The product, 4-methylumbelliferyl, shows a peak at 440nm in the fluorescence spectra.
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Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, type N95 (US)
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Omid Motabar et al.
Analytical biochemistry, 390(1), 79-84 (2009-04-18)
Mutations in alpha-glucosidase cause accumulation of glycogen in lysosomes, resulting in Pompe disease, a lysosomal storage disorder. Small molecule chaperones that bind to enzyme proteins and correct the misfolding and mistrafficking of mutant proteins have emerged as a new therapeutic
Intrapleural administration of AAV9 improves neural and cardiorespiratory function in Pompe disease.
Darin J Falk et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 21(9), 1661-1667 (2013-06-05)
Pompe disease is a neuromuscular disease resulting from deficiency in acid α-glucosidase (GAA), results in cardiac, skeletal muscle, and central nervous system (CNS) pathology. Enzyme replacement therapy (ERT) has been shown to partially correct cardiac and skeletal muscle dysfunction. However
Phillip A Doerfler et al.
Human gene therapy, 27(1), 43-59 (2015-11-26)
Pompe disease is a progressive neuromuscular disorder caused by lysosomal accumulation of glycogen from a deficiency in acid alpha-glucosidase (GAA). Replacement of the missing enzyme is available by repeated protein infusions; however, efficacy is limited by immune response and inability
Global Trade Item Number
| SKU | GTIN |
|---|---|
| M9766-100MG | 04061834064558 |
| M9766-250MG | 04061835546190 |
| M9766-1G | 04061835557776 |
| M9766-10MG | 04061834064565 |
| M9766-25MG | 04061835557783 |