Abnormalities of Carbamyl Phosphate Synthetase and Ornithine Transcarbamylase in Liver of Patients with Reye's Syndrome

Abstract

Pediat. R e s . 9: 829-833 (1975) C a r b a m y l phosphate synthetase Reye's s y n d r o m e ornithine transcarbamylase u r e a cycle

Urinary purines and pyrimidines in patients with hyperammonemia of various origins

“…did not correlate with plasma ammonia. In Reye's syndrome, the hepatic mitochondrial enzymes CPS-I and OCT have been found to be drastically reduced (at least during the first days of the clinical symptoms), the cytoplasmatic enzymes of the urea cycle being normal [ 11,[45][46][47][48].…”

Deficient Activity of Hepatic Pyruvate Dehydrogenase and Pyruvate Carboxylase in Reye's Syndrome

“…Defective urea cycle function in a damaged liver leading to hyperammonemia has been suggested as central to the pathogenesis of encephalopathy in Reye's syndrome (18,19,22), although it has now been shown that the activities of hepatic ornithine transcarbamylase and carbamyl phosphate synthetase slowly return to normal after the acute phase of the disease (7,18). This is so whether or not the progression of the disease leads to fatality (7).…”

“…These findings correlate with observations of decreased activity in two urea cycle enzymes present in liver mitochondria, ornithine transcarbamylase (EC. 2.1.3.3) (7,18,20,22) and carbamyl phosphate synthetase (EC. 2.7.2.5) (7,18).…”

“…2.1.3.3) (7,18,20,22) and carbamyl phosphate synthetase (EC. 2.7.2.5) (7,18). Histochemical observation of succinate dehydrogenase (EC.…”

Biochemical heterogeneity of ornithine carbamoyl transferase(OCT) in patients with OCT deficiency

“…Most studies carried out so far were attempts to clarify the heterogeneity of mutant OCT according to the kinetic characteristics (Matsuda et al, 1971;Cathelineau et al, 1974;Sinatora et al, 1975;Snodograss, 1981). Francois et al (1982) and Mori et al (1980) extended studies on mutant OCT analysis by use of an immunochemical method.…”