Table 1. Synthesis of 11cROL (pmol/mg protein).
Retinoid isomerase activities of WT and the indicated mutant RPE65s were determined by measuring synthesis of 11-cis retinol (11cROL) at 30°C or 37°C. Numbers indicate 11cROL content (pmol ± SD, n = 3) in 1 mg of cellular protein (middle columns) or ratio of the isomerase activities at 30°C to those at 37°C (right column). NA, no activity.
Mutation | Activity at 30°C | Activity at 37°C | Ration 30/37°C |
---|---|---|---|
L22P | 22 ± 3 | 4 ± 1 | 5.5 |
T101I | 12 ± 2 | 2 ± 1 | 6.0 |
L408P | 26 ± 3 | 5 ± 1 | 5.2 |
H180R | NA | NA | – |
H313R | NA | NA | – |
Y239D | 1.3 ± 0.3 | 1.3 ± 0.5 | 1.0 |
C330Y | 1.8 ± 0.3 | 1.6 ± 0.3 | 1.1 |
E417Q | 1.2 ± 0.4 | 1.1 ± 0.4 | 1.1 |
WT | 124 ± 10 | 138 ± 10 | 0.9 |