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- W2001798006 abstract "Biochemical studies were performed on two siblings with pseudo-Hurler polydystrophy (ML III). Although mucopolysaccharide excretion was not in excess, the levels tended toward the upper limit of the normal range. Electrophoretic studies revealed abnormal glycoprotein components in the urine of both patients. Leukocyte extracts had a higher than normal content of neutral sugar, sialic acid and hexosamine, suggesting the presence of excess glycoprotein(s). The most striking abnormalities were in the acid hydrolase activities of cultured fibroblasts. Affected cells had essentially no detectable α-l-fucosidase, and the activities of seven other glycosidases ranged from only 11 to 37% of normal controls. The acid phosphatase activity in the fibroblast cultures was normal, while β-glucosidase activity was more than twice the mean level in normal control cultures. Concomitant with the marked decrease in cellular activities of eight of the ten acid hydrolases studied, an excess of nearly all of these enzymes was found in the culture medium of the ML III patient. The similarities in biochemical and other abnormalities in ML III to those reported for ML II (I-cell disease) are discussed." @default.
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- W2001798006 date "1974-04-01" @default.
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- W2001798006 title "Acid hydrolase deficiencies and abnormal glycoproteins in mucolipidosis III (pseudo-Hurler polydystrophy)" @default.
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- W2001798006 doi "https://doi.org/10.1016/0009-8981(74)90394-5" @default.
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