Glucose tetrasaccharide (Glc4) is used as a reference in analysis of urinary glucose tetrasacchaide, a biomarker for Pompe disease and other glycogen storage diseases.
Packaging
Bottomless glass bottle. Contents are inside inserted fused cone.
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Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme, acid alpha-glucosidase (GAA). To the best of our knowledge, no studies have reported the results of systematic and sequential CT analyses before and during ERT. In
Journal of immunological methods, 48(1), 109-119 (1982-01-01)
A radioimmunoassay is described that allows rapid determination of a urinary oligosaccharide -- Glc alpha 1-6Glc alpha 1-4Glc alpha 1-4Glc [(Glc)4] -- at concentrations greater than 2 pmol/microliter. Antibodies produced in rabbits immunized with the phenethylamine derivative of (Glc)4 coupled
Journal of inherited metabolic disease, 35(2), 311-316 (2011-06-21)
A high performance liquid chromatography method, adapted from an established urinary sugars method, has been developed for the analysis of a tetraglucose oligomer (Glc(4)) in urine. Pompe disease results from defects in the activity of lysosomal acid α-glucosidase (GAA) with
Molecular genetics and metabolism reports, 23, 100591-100591 (2020-05-07)
Enzyme replacement therapy (ERT), the only approved therapy for infantile-onset Pompe disease (IOPD), had heterogeneous clinical effects due to factors such as severity, age at first treatment, dosage, and dosing regimens. We report the clinical and biochemical outcomes of a
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