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The method of claim 4, wherein the sphingolipidosis is Gaucher disease and the lysosomal enzyme is Glucosylceramide ?-glucosidase

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In vivo stability Collected urine was measured in PBS (10 mM, pH 7.4) by UV-Vis and fluorescence spectrophotometry, imaged with the IVIS spectral imaging system, and analyzed by HPLC as well as MALDI-TOF mass spectrometry