Increased sensitivity of cerebrohepatorenal syndrome fibroblasts to antimycin A.

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.