| 6321855 |
Kelley RI, Corkey BE: Increased sensitivity of cerebrohepatorenal syndrome fibroblasts to antimycin A. J Inherit Metab Dis. 1983;6(4):158-62.
The effect of four mitochondrial electron transport inhibitors on the growth of normal and cerebrohepatorenal syndrome (CHRS) fibroblasts was studied. Compared to normal fibroblasts, CHRS cells demonstrated a fivefold greater sensitivity to antimycin A, an inhibitor of the cytochrome bc1 complex (complex III), but equal inhibition by amytal, rotenone and 2-heptyl-4-hydroxyquinoline-N-oxide. The results suggest a primary or secondary functional deficiency at mitochondrial complex III in fibroblasts. |
32(0,1,1,2) |