نتایج جستجو برای: urea cycle disorders
تعداد نتایج: 967162 فیلتر نتایج به سال:
Urea cycle disorders in Spain: an observational, cross-sectional and multicentric study of 104 cases
Maloran, 3-(3-chloro, 4-bromophenyl)-1-methoxy-lmethylurea, is a selective herbicide nematicide used for weed control in corn. The substituted urea herbicides as a group are degraded in the soil by microbial action (3,4,7) with minimal accumulation of phytotoxic residues. Geissbuhler et al. (3) showed that cluroxuron, 3-(p chlorophenoxyphenyl)-1, 1-dimethylurea was degraded in soil by successi...
Mono-urea-functionalized pillar[5]arenes were prepared which exhibited abnormal urea behaviors. They were shown to form pseudo[1]rotaxanes with poor anion binding abilities in solution and [c2]daisy chains with an abnormal urea motif in the solid state.
Neonatal-onset propionic acidemia is the most common form of disorder. A 9-days old new born admitted to our hospital with lethargy suggested urea cycle defect at first look due to lack of metabolic acidosis, normal ketone and anion gap in laboratory evaluations. The case mimicking urea cycle defect, which was diagnosed as neonatal-onset propionic acidemia by specific tests, was presented becau...
N-acetylglutamate synthase (NAGS) deficiency is a rare inborn error of metabolism affecting ammonia detoxification in the urea cycle. The product of NAGS is N-acetylglutamate which is the absolutely required allosteric activator of the first urea cycle enzyme carbamoylphosphate synthetase 1. In defects of NAGS, the urea cycle function can be severely affected resulting in fatal hyperammonemia i...
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