Biochemical assay of fibroblasts from complementation group A (CG-A) patients
| No. | Type | C24/C16 | DHAP-AT | ||||||
|---|---|---|---|---|---|---|---|---|---|
| 37°C | 30°C | 37°C | 30°C | ||||||
| A-06 | ZS | 0.040 | 0.069 | 0.09 | 0.15 | ||||
| A-14 | ZS | 0.011 | 0.042 | 0.18 | 0.30 | ||||
| A-10 | ZS | 0.007 | 0.030 | 0.27 | 0.46 | ||||
| A-05 | NALD | 0.077 | 0.199 | 0.39 | 1.65 | ||||
| A-08 | NALD | 0.013 | 0.287 | 0.19 | 1.55 | ||||
| Control | 0.522 | 0.478 | 1.53 | 1.90 | |||||
Fibroblasts were cultured for eight days at either 37°C or 30°C, and then assayed for beta oxidation activities of lignoceric (C24) and palmitic acids (C16) and the ratio of C24/C16 was calculated. DHAP-AT activity was measured in each cell line. These results were the average of duplicates. DHAP-AT activity was indicated as nmol/mg/120 min. The methods have been described in Suzuki et al 20 and Shimozawa et al.21