Clinical and investigative medicine. Medecine clinique et experimentale, 13(4), 183-188 (1990-08-01)
Liver tissue from 3 patients with carbamyl phosphate synthetase I deficiency and 11 patients with ornithine transcarbamylase deficiency was analyzed by Western blots for the presence of carbamyl phosphate synthetase I and ornithine transcarbamylase cross-reactive material with anti-rat enzyme antibodies.
Biochemical medicine and metabolic biology, 47(1), 38-46 (1992-02-01)
N-Acetyl-L-glutamate synthetase (NAG synthetase) is a mitochondrial matrix enzyme which catalyzes the synthesis of N-acetyl-Lglutamate (NAG), a physiologic activator of the urea cycle enzyme carbamylphosphate synthetase I. Deficiency of NAG synthetase in humans has been reported only three times previously.
N-acetylglutamate (NAG) content was measured in homogenates of liver and small intestine obtained from normal and 24-h starved syngeneic mice. Subsequently, NAG was determined in normal, and in carbamyl phosphate synthetase I and ornithine transcarbamylase enzyme-deficient human liver tissue homogenates.
Journal of chromatography. B, Biomedical applications, 657(1), 15-21 (1994-07-01)
Liquid chromatography-atmospheric pressure chemical-ionization mass spectrometry (LC-APCI-MS) was used for the analysis of N-acetylamino acids that could not be determined with an amino acid analyzer. LC-APCI-MS could directly detect the protonated molecular ions of various synthetic N-acetylamino acids, distinguishing N-acetylserine
A sensitive and reliable radioimmunoassay (RIA) for urinary unconjugated 5 alpha-androstane-3 alpha, 17 beta-diol is described. The mean overall recovery of unconjugated 5 alpha-androstane-3 alpha, 17 beta-diol was found to be 57.4%. The sensitivity of the assay was 79 fmol
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