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- W2088391438 abstract "Abstract The activities of most lysosomal enzymes are elevated in the culture fluid of skin fibroblasts derived from patients with I-cell disease with a corresponding reduction in the intracellular activities when the cells are cultured at 37°C. When I-cell fibroblast cultures are incubated at 27°C for 8–24 hr, the β-D-N-acetylglucosaminidase (EC 3.2.1.30) activity accumulated in the culture fluid is reduced to approximately 25% of the activity in 37°C control cultures without a corresponding change in intracellular activity. No significant effect of temperature is observed on the intra- and extracellular distribution of β-D-N-acetylglucosaminidase in non-I-cell fibroblast cultures. These findings suggest the existence of two lysosomal enzyme pools in I-cell fibroblasts, one of which is temperature-dependent and destined for excretion while the other remains intracellular and appears to be unaffected by temperature." @default.
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- W2088391438 date "1979-06-01" @default.
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- W2088391438 title "Effect of temperature on extracellular accumulation of β-D-N-acetylglucosaminidase in I-cell disease fibroblast cultures" @default.
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- W2088391438 doi "https://doi.org/10.1016/0024-3205(79)90535-6" @default.
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