Propionate mitochondrial toxicity in liver and skeletal muscle: acyl CoA levels
T Matsuishi, DA Stumpf, M Seliem, LA Eguren… - … medicine and metabolic …, 1991 - Elsevier
Propionic acidemia occasionally produces a toxic encephalopathy resembling Reye
syndrome, indicating disruption of mitochondrial metabolism. Understanding the
mitochondrial effect of propionate might clarify the pathophysiology. Liver mitochondria are
inhibited by propionate (5 mm) while muscle mitochondria are not. Preincubation is required
to inhibit liver mitochondria, suggesting that propionate is metabolized to propionyl CoA.
Liver and skeletal muscle mitochondria incubated with [1-14 C] propionate contain similar …
syndrome, indicating disruption of mitochondrial metabolism. Understanding the
mitochondrial effect of propionate might clarify the pathophysiology. Liver mitochondria are
inhibited by propionate (5 mm) while muscle mitochondria are not. Preincubation is required
to inhibit liver mitochondria, suggesting that propionate is metabolized to propionyl CoA.
Liver and skeletal muscle mitochondria incubated with [1-14 C] propionate contain similar …