Rayment, "Structure of carbamoyl phosphate
synthetase: a journey of 96 a from substrate to product," Biochemistry, vol.
The antigen for Hep Par 1 antibody is the urea cycle enzyme carbamoyl phosphate synthetase 1.
Intestinal carbamoyl phosphate synthase I in human and rat: expression during development shows species differences and mosaic expression in duodenum of both species.
All the natural phospho-donors (acetyl phosphate, carbamoyl phosphate
, phosphoramidate, and monophosphoimidazole) bind to active site with binding energy ranging from -3.32 to -3.93 kcal/mol and inhibition constant from 1.31 mM to 3.67 mM (Table 1).
OA detection in urine forms a useful surrogate marker of increased carbamoyl phosphate
, a substrate of OTC (Fig.
OTCD patients have low blood citrulline because of reduced conversion from carbamoyl phosphate
. Citrulline is one of the amino acids routinely measured in MS/MS newborn-screening programs.