Comparison of cytosolic and mitochondrial enzyme alterations in the livers of propionic or methylmalonic acidemia: A reduction of cytochrome oxidase activity.

Publisher Website
Google Scholar
Abstract
The activities of mitochondrial, cytosolic and microsomal enzymes in liver specimens obtained from 3 patients with propionic or methylmalonic acidemia were compared with those of control patients who had died from unrelated causes. Only the activity of cytochrome oxidase (mitochondrial enzyme) was significantly reduced in patients with propionic acidemia and methylmalonic acidemia who were in a state of metabolic acidosis; in 2 patients the activity was less than 30% of that in controls, but in the other patients with propionic acidemia, who were under treatment with a low protein diet (0.8 g/kg per day), the activity was 50% of that in controls. The metabolites of branched chain amino acids (tiglic acid, propionic acid, methylmalonic acid, succinic acid, tiglyl-CoA and propionyl CoA) exhibited no inhibitory effect on the cytochrome oxidase activity of sonicated rat liver mitochondria. The reduction of cytochrome oxidase activity found in these organic acidemias may be caused secondarily by some unknown mechanism.

This publication has 1 reference indexed in Scilit: