PMID: 36157Apr 12, 1979

Use of immobilized antibodies in investigating acid alpha-glucosidase in urine in relation to Pompe's disease

Biochimica Et Biophysica Acta
A W SchramJ M Tager


(1) A simple method is described for the isolation of the lysosomal enzyme, acid alpha-glucosidase (alpha-D-glucoside glucohydrolase, EC from normal human liver. Antibodies raised against the purified enzyme were immobilized by covalent coupling to Sepharose 4B. (2) Acid alpha-glucosidase can be quantitatively removed from normal urine by incubating with an excess of immobilized antibody. With p-nitrophenyl-alpha-glucoside as substrate, acid alpha-glucosidase accounts for 91 +/- 3% of the total alpha-glucosidase activity at pH 4.0 IN Normal urine. (3) In urine from a patient with the infantile form of Pompe's disease ('acid maltase deficiency'), no alpha-glucosidase activity could be removed by the immobilized antibody, in agreement with the fact that acid alpha-glucosidase is absent in these patients. (4) In urine from patients with the late-onset form of Pompe's disease, 46 +/- 11% of the alpha-glucosidase activity at pH 4.0 can be removed by incubation with immobilized antibodies, indicating that residual acid alpha-glucosidase activity is present in urine of these patients. The residual acid alpha-glucosidase activity amounts to about 5% of that in the urine of control persons. (5) If acid alpha-glucosidase is ads...Continue Reading


Jul 30, 1986·Clinica Chimica Acta; International Journal of Clinical Chemistry·J M AertsA W Schram
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Jan 1, 1994·Journal of Child Neurology·C Y TsaoF S Wright
Mar 15, 1984·European Journal of Biochemistry·R Oude ElferinkJ M Tager
Jan 1, 1981·Clinical Genetics·M C LoonenJ M Tager


Mar 1, 1978·The Journal of Clinical Investigation·K Paigen, J Peterson
Jan 1, 1978·Muscle & Nerve·S DiMauroC Payne
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Nov 1, 1971·The Journal of Pediatrics·I S Salafsky, H L Nadler
Feb 1, 1973·The Journal of Pediatrics·I S Salafsky, H L Nadler
Mar 1, 1973·Archives of Biochemistry and Biophysics·J Carter, E E Smith
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Related Concepts

Lysosomal alpha-Glucosidase
Glycogen Storage Disease Type II
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Peroxidase-Antiperoxidase Complex Technique
Generalized Glycogen Storage Disease of Infants

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