Serum ornithine carbamyl transferase (OCT) was evaluated on eighteen dogs. Three groups were used, the first and second ones were treated with carbon tetrachloride (CCI) to produce acute hepatic necrosis and cirrhosis. To the third group a ductus choledochus ligation was performed to simulate extrahepatic cholestasis. Ornithine carbamyl transferase has proven its use in all phases of hepatic necrosis, but only after the fourth day in the extrahepatic cholestasis. Finally in cirrhosis, it was sometimes difficult to interpret the results.
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J Inherit Metab Dis
March 2024
Department of Paediatrics I, Medical University of Innsbruck, Innsbruck, Austria.
Carbamoyl phosphate synthetase 1 (CPS1) and ornithine transcarbamylase (OTC) deficiencies are rare urea cycle disorders, which can lead to life-threatening hyperammonemia. Liver transplantation (LT) provides a cure and offers an alternative to medical treatment and life-long dietary restrictions with permanent impending risk of hyperammonemia. Nevertheless, in most patients, metabolic aberrations persist after LT, especially low plasma citrulline levels, with questionable clinical impact.
View Article and Find Full Text PDFZhejiang Da Xue Xue Bao Yi Xue Ban
October 2023
Department of Genetics and Metabolism, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
Urea cycle disorder (UCD) is a group of inherited metabolic diseases with high disability or fatality rate, which need long-term drug treatment and diet management. Except those with Citrin deficiency or liver transplantation, all pediatric patients require lifelong low protein diet with safe levels of protein intake and adequate energy and lipids supply for their corresponding age; supplementing essential amino acids and protein-free milk are also needed if necessary. The drugs for long-term use include nitrogen scavengers (sodium benzoate, sodium phenylbutyrate, glycerol phenylbutyrate), urea cycle activation/substrate supplementation agents (-carbamylglutamate, arginine, citrulline), etc.
View Article and Find Full Text PDFAm J Physiol Gastrointest Liver Physiol
October 2023
Robert Wood Johnson Medical School, Rutgers University, New Brunswick, New Jersey, United States.
Carbamoyl phosphate synthetase 1 (CPS1) is the most abundant hepatocyte mitochondrial matrix protein. Hypoosmotic stress increases CPS1 release in isolated mouse hepatocytes without cell death. We hypothesized that increased CPS1 release during hypoosmosis is selective and associates with altered mitochondrial morphology.
View Article and Find Full Text PDFPLoS One
September 2022
Research Unit of Cryogenic Novel Material, Korea Polar Research Institute, Incheon, Republic of Korea.
Ornithine carbamoyltransferases (OTCs) are involved in the arginine deiminase (ADI) pathway and in arginine biosynthesis. Two OTCs in a pair are named catalytic OTC (cOTC) and anabolic OTC (aOTC). The cOTC is responsible for catalyzing the third step of the ADI pathway to catabolize citrulline into carbamoyl phosphate (CP), as well as ornithine, and displays CP cooperativity.
View Article and Find Full Text PDFJ Clin Lab Anal
October 2022
Department of Biochemistry and Molecular Biology, School of Preclinical Medicine and Forensic Medicine, North Sichuan Medical College, Nanchong, Sichuan Province, China.
Objective: The sensitivity and specificity of current biomarkers for gastric cancer were insufficient. The aim of the present study was to screen novel biomarkers and determine the diagnostic values of ornithine aminotransferase (OAT) and carbamoyl phosphate synthetase 1 (CPS1) for detecting gastric cancer.
Methods: With stable isotope tags, we labelled an initial discovery group of four paired gastric cancer tissue samples and identified with LC-ESI-MS/MS.
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