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Increased sensitivity of cerebrohepatorenal syndrome fibroblasts to antimycin A
Author(s) -
Kelley R. I.,
Corkey B. E.
Publication year - 1983
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf02310872
Subject(s) - antimycin a , rotenone , mitochondrion , fibroblast , coenzyme q – cytochrome c reductase , cytochrome , electron transport chain , chemistry , microbiology and biotechnology , biology , cytochrome c , biochemistry , genetics , enzyme , cell culture
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 bc 1 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.