3-Hydroxy-3-methylglutaryl coenzyme A reductase activity in cultured fibroblasts from patients with mevalonate kinase deficiency: differential response to lipid …
KM Gibson, G Hoffmann, A Schwall, RL Broock… - Journal of lipid …, 1990 - ASBMB
3-Hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase activity was measured in
extracts of cultured fibroblasts derived from patients with mevalonate kinase deficiency
(MKD). For six patients studied, the mean activity of 63.3+/-41.1 pmol/min-mg protein (+/-1
SD, range 37.7-146.2) was significantly higher than the mean value in three control
fibroblast lines of 11.1+/-3.5 (+/-1 SD, range 8.0-14.9). These values were obtained using
cells subcultured in medium supplemented with 10% fetal bovine serum (FBS) 21 h prior to …
extracts of cultured fibroblasts derived from patients with mevalonate kinase deficiency
(MKD). For six patients studied, the mean activity of 63.3+/-41.1 pmol/min-mg protein (+/-1
SD, range 37.7-146.2) was significantly higher than the mean value in three control
fibroblast lines of 11.1+/-3.5 (+/-1 SD, range 8.0-14.9). These values were obtained using
cells subcultured in medium supplemented with 10% fetal bovine serum (FBS) 21 h prior to …