TABLE 2.
Acid ceramidase activity in normal and Farber disease cell lines
| FIBROBLASTS | LYMPHOID CELLS | ||||||
|---|---|---|---|---|---|---|---|
| aCDase (pmol/h/mg) | lysosomal β Gal (nmol/h/mg) | aCDase/lysosomal β Gal | aCDase (pmol/h/mg) | lysosomal β Gal (nmol/h/mg) | aCDase/lysosomal β Gal | ||
| CTRL1 | 5816 ± 553 | 1546 ± 38 | 3.761 | CTRL5 | 8396 ± 1675 | 260 ± 25 | 32.32 |
| CTRL2 | 3785 ± 404 | 2114 ± 370 | 1.790 | CTRL6 | 14367 ± 2781 | 516 ± 42 | 27.86 |
| CTRL3 | 5628 ± 1337 | 767 ± 53 | 7.330 | CTRL7 | 15625 ± 4725 | 468 ± 114 | 33.36 |
| CTRL4 | 9388 ± 2731 | 2827 ± 333 | 3.321 | CTRL8 | 8416 ± 2892 | 420 ± 59 | 20.06 |
| FD1 | −32 ± 76 | 2726 ± 1139 | −0.012 | FD6 | 235 ± 153 | 480 ± 105 | 0.49 |
| FD2 | −1 ± 115 | 2145 ± 489 | −0.001 | FD7 | 241 ± 211 | 395 ± 233 | 0.61 |
| FD3 | 45 ± 125 | 2634 ± 162 | 0.017 | FD8 | 539 ± 369 | 605 ± 305 | 0.94 |
| FD4 | 55 ± 74 | 1815 ± 695 | 0.031 | FD9 | 707 ± 438 | 664 ± 121 | 0.66 |
| FD5 | −18 ± 13 | 846 ± 311 | −0.022 | FD10 | 200 ± 76 | 268 ± 181 | 0.74 |
aCDase activity was determined using Rbm14-12 at 20 µM after incubation for 3 h with lysates (20 µg protein) from fibroblasts or lymphoid cells derived from control individuals (CTRL) and patients affected with FD. As a control enzyme, β-galactosidase activity was determined. Results are the average of three independent experiments.