Abstract
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.
Original language | English (US) |
---|---|
Pages (from-to) | 158-162 |
Number of pages | 5 |
Journal | Journal of Inherited Metabolic Disease |
Volume | 6 |
Issue number | 4 |
DOIs | |
State | Published - Dec 1983 |
Externally published | Yes |
ASJC Scopus subject areas
- Genetics
- Genetics(clinical)