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. 2009 Nov 13;85(5):569–580. doi: 10.1016/j.ajhg.2009.09.016

Table 1.

Expression Study on the NAGA and Modified NAGA cDNAs

Cells Introduced cDNA MU-α-D-Galactopyranoside-Degrading Activity (nmol/h/mg protein) MU-α-D-N-Acetylgalactosamine-Degrading Activity (nmol/h/mg protein)
Wild type - 30 2.5 × 102
Kanzaki - 40 <1.0
Fabry - 2.0 2.3 × 102
NAGA 13 1.3 × 104
modified NAGA 5.5 × 102 1.0 × 102

Wild type, human cultured fibroblasts from an apparently normal subject; Kanzaki, cultured fibroblasts from a patient with Kanzaki disease (inherited NAGA deficiency); and Fabry, cultured fibroblasts from a patient with Fabry disease.