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Targeted therapy! The Capetin Prize winning "Click Chemistry" can be used like this!Targeted therapy! The Nobel Prize winning "Click Chemistry" can be used like this!2025-02-06
9 Results for "
Pompe disease
" in MedChemExpress (MCE) Product Catalog:
Products (9)
9 Results for "Pompe disease" in MCE Product Catalog:
4
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Research Areas:
MZ-101 (GYS1-IN-2) is an orally active, potent and selective small-molecule glycogen synthase 1 (GYS1) inhibitor with with an IC50 value of 0.041 µM. MZ-101 reduces glycogen concentrations in cells and in mice. MZ-101 can used to study GYS1 -mediated Pompe disease and other glycogen storage diseases .
Synonyms: 4-Methylumbelliferyl-α-D-Glucose; 4-Methylumbelliferyl-α-D-Glucoside; 4-MU-α-D-Glucopyranoside
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4-Methylumbelliferyl-α-D-Glucopyranoside (4-Methylumbelliferyl-α-D-Glucose) is a fluorescent substrate for α-glucosidase, which releases the fluorescent moiety 4-methylumbelliferyl (4-MU) upon cleavage. 4-MU has pH-dependent fluorescence excitation activity, with excitation wavelengths of 320 nm at low pH (1.97-6.72) and 360 nm at high pH (7.12-10.3), respectively. The emission wavelength of 4-Methylumbelliferyl-α-D-Glucopyranoside increases with decreasing pH, ranging from 445-455 nm. 4-Methylumbelliferyl-α-D-Glucopyranoside can be used as a biomarker for Fabry and Pompe diseases to quantify α-glucosidase activity in infant blood spot samples.
Cat. No.: HY-E70183
Synonyms: EC:3.2.1.20; GAA
Cat. No.: HY-178202
CAS No.: 2734265-58-6
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5-C-phenethyl-DNJ is a selective α-glucosidase GAA inhibitor, with its Ki value for rhGAA being 0.81 μM. 5-C-phenethyl-DNJ exhibits extremely high selectivity for GANAB, GBA1, and GBA2. 5-C-phenethyl-DNJ can be used for the study of Pompe disease .
Cat. No.: HY-100127
CAS No.: 1222374-44-8
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ML247 is a non-inhibitory and selective small molecule chaperone of acid alpha glucosidase. ML247 can be used for the research of metabolic disease, such as Pompe disease .
Cat. No.: HY-P99803
CAS No.: 2145123-44-8
Synonyms: VAL-1221
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Cat. No.: HY-108828
CAS No.: 420784-05-0
Synonyms: rhGAA
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Alglucosidase alfa (rhGAA) is a recombinant human acid α-glucosidase. Alglucosidase alfa is taken up by cells via the cation-independent mannose-6-phosphate receptor (CI-MPR) pathway and transported to lysosomes, where GAA degrades glycogen in the acidic lysosomal environment. Alglucosidase alfa is applicable to research related to lysosomal glycogen metabolism and glycogen storage diseases .
Cat. No.: HY-178200
CAS No.: 2763641-30-9
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5-C-phenethyl-DNJ hydrochloride is an orally active acid α-glucosidase (GAA) activator. 5-C-phenethyl-DNJ hydrochloride can increase GAA activity in various tissues, particularly in the diaphragm. 5-C-phenethyl-DNJ hydrochloride can be used for the research of metabolic disease, such as Pompe disease .
Cat. No.: HY-P810401
Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid (Pompe disease glycogen storage disease type II), Glucosidase alpha acid, Glucosidase alpha, LYAG
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Protocols
Targeted therapy! The Capetin Prize winning "Click Chemistry" can be used like this!Targeted therapy! The Nobel Prize winning "Click Chemistry" can be used like this!A suitable model is crucial in drug screening experiments. Organs can mimic the three-dimensional functional structure of internal organs, have similar spatial organization to corresponding organs, maintain some key characteristics, and reproduce some physiological functions. They are widely used for modeling and personalized drug screening physiological functions.2025-02-06
