A simple differential immunoprecipitation assay of urinary acid and neutral alpha-glucosidases for glycogenosis II.
A specific assay for acid alpha-glucosidase in urine was developed to facilitate the diagnosis of glycogenosis II. This enzyme activity was calculated as a difference between the alpha-glucosidase activities before and after immunoprecipitation with antiserum to acid alpha-glucosidase. Acid alpha-glucosidase accounted for 86% of the total activity in control urine. All the cases of various clinical types of glycogenosis II showed either a marked decrease or a complete deficiency of this enzyme activity. A marked decrease of acid alpha-glucosidase was demonstrated by immunoblotting of the urine from patients with late-onset forms of this disease. These results indicate that assays of urinary acid alpha-glucosidase by this immunological method are useful for detection of the various types of glycogenosis II.
Tsuji
A
A
Yang
R C
RC
Omura
K
K
Imabayashi
T
T
Suzuki
Y
Y
D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006008:Glycogen Storage Disease / Q000175:diagnosis*; D006009:Glycogen Storage Disease Type II / Q000175:diagnosis* / Q000201:enzymology; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007118:Immunoassay; D008297:Male; D011247:Pregnancy; D000520:alpha-Glucosidases / Q000652:urine*
D000520:alpha-Glucosidases