Lysosomal tartrate sensitive acid phosphatase deficiency in cells which contain lysosomal "high uptake forms".
The catalytic and immunological properties of acid phosphatases (EC 3.1.3.2.) in different tissues were studied. It was demonstrated that high uptake forms of lysosomal enzymes like beta-galactosidase isolated from human platelets and bovine testis are mature enzymes, which have not lost their mannose-6phosphate marker. The results presented indicate that this phenomenon is related to a low activity or the complete absence of the lysosomal tartrate sensitive acid phosphatase activity in the tissues concerned.
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Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.
Parenti
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Keulemans
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Hoogeveen
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AT
D000135:Acid Phosphatase / Q000037:antagonists & inhibitors / Q000172:deficiency / Q000378:metabolism*; D000818:Animals; D001792:Blood Platelets / Q000201:enzymology; D002417:Cattle; D002460:Cell Line; D002478:Cells, Cultured; D005260:Female; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007118:Immunoassay; D007700:Kinetics; D008247:Lysosomes / Q000201:enzymology*; D008264:Macrophages / Q000201:enzymology; D008297:Male; D008970:Molecular Weight; D010920:Placenta / Q000201:enzymology; D011247:Pregnancy; D013644:Tartrates / Q000494:pharmacology*; D013737:Testis / Q000201:enzymology
D013644:Tartrates; D000135:Acid Phosphatase