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Lymphocyte -Glucosidase in Late-Onset Glycogenosis Type II

Masaru Kuriyama, MD; Tatsuo Kohriyama, MD; Teruo Iwamasa, MD; Ryo-ichi Hiwatari, MD; Mitsuhiro Osame, MD; Akihiro Igata, MD

Arch Neurol. 1989;46(4):460-462.

Abstract
• We describe the biochemical characterization of lymphocyte -glucosidase in a 23-year-old man with intermediate clinical features between the childhood and adult forms of glycogenosis type II (Pompe's disease). Acid -glucosidase activity was markedly reduced, but immunologic cross-reactive material against human liver acid -glucosidase protein could be detected, and its amount was normal. In this patient, the disorder was induced by the catalytically inactive enzyme with a normal amount of enzyme protein.

Author Affiliations
From the Third Department of Internal Medicine, Kagoshima (Japan) University School of Medicine (Drs Kuriyama, Kohriyama, Hiwatari, Osame, and Igata), and the Department of Pathology, Ryukyu University School of Medicine, Okinawa, Japan (Dr Iwamasa).

Footnotes
Accepted for publication April 12, 1988.

Reprint requests to Third Department of Internal Medicine, Kagoshima University School of Medicine, 1208-1 Usuki-cho, Kagoshima 890, Japan (Dr Kuriyama).

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