The molecular heterogeneity of purified human liver lysosomal α-Glucosidase (acid α-Glucosidase)

Characterization of the molecular defect in infantile and adult acid alpha-glucosidase deficiency fibroblasts.

“…A similar competition for antibody binding sites also was shown between normal and adult acid a-glucosidase deficiency fibroblast enzymes. The apparently contradictory findings of this study with the earlier reported absence or severe reduction of immunologically reacting enzyme protein in tissues of patients with glycogenosis type II (11)(12)(13)(14)27) could be interpreted as the result of genetic heterogeneity.…”

Acid and neutral -glucosidase in the reproductive organs and seminal plasma of the bull

“…Martiniuk & Hirschhorn (1981), on the other hand, showed that two neutral a-glucosidases (called AB and C) from human tissues had isoelectric points of 61 and 5-5, respectively. In the human liver several forms of acid -glucosidase with pi values between 4-4 and 4-7 were also demonstrated by Murray, Brown & Brown (1978). At least partly these differences can be ascribed to modifications in the carbohydrate side-chains of their glycoprotein structure.…”

Demonstration of acid maltase protein in Pompe disease by use of immunohistochemical and enzyme immunoassay methods

“…(1978) reported that the enzyme protein in the infantile form of Pompe disease could be demonstrated by the immunodiffusion test on an agar plate. On the other hand, a few reports have shown no detectable enzyme protein or immunological cross reactive material against anti-human acid maltase antibody by use of immunodiffusion on an agar plate (Murray et al, 1978;deBarsy et al, 1972;Koster and Slee, 1977). In this study, the muscle of five cases of Pompe disease-two cases of infantile type, one of childhood type and two of adult type-were investigated immunohistochemically and by use of the enzyme immunoassay method.…”