Table 1.
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.