Indexes of citrulline metabolism in rat liver under the toxic injury against the background of alimentary protein deficiency [PDF]
The Ukrainian Biochemical Journal, 2020 It is known that citrulline is converted into arginine in the series of metabolic transformations. Results of our previous studies showed that acetaminophen-induced toxic injury on the background of the alimentary deprivation of protein is accompanied by
H. P. Kopylchuk +2 more
doaj +1 more source
Effects of Dufulin on Oxidative Stress and Metabolomic Profile of Tubifex
Metabolites, 2021 Dufulin is a highly effective antiviral pesticide used in plants. In this study, a seven-day experiment was conducted to evaluate the effects of Dufulin at five different concentrations (1 × 10−4, 1 × 10−3, 1 × 10−2, 0.1, and 1 mg/L) on Tubifex.
Yile Yu +5 more
doaj +1 more source
ASS1 and ASL suppress growth in clear cell renal cell carcinoma via altered nitrogen metabolism
Cancer & Metabolism, 2021 Background Kidney cancer is a common adult malignancy in the USA. Clear cell renal cell carcinoma (ccRCC), the predominant subtype of kidney cancer, is characterized by widespread metabolic changes.
Sanika Khare +7 more
doaj +1 more source
Argininosuccinate lyase is a metabolic vulnerability in breast development and cancer
npj Systems Biology and Applications, 2021 Epithelial-to-mesenchymal transition (EMT) is fundamental to both normal tissue development and cancer progression. We hypothesized that EMT plasticity defines a range of metabolic phenotypes and that individual breast epithelial metabolic phenotypes are
Sigurdur Trausti Karvelsson +8 more
doaj +1 more source
Beclin‐1‐mediated activation of autophagy improves proximal and distal urea cycle disorders
EMBO Molecular Medicine, 2020 Urea cycle disorders (UCD) are inherited defects in clearance of waste nitrogen with high morbidity and mortality. Novel and more effective therapies for UCD are needed.
Leandro R Soria +18 more
doaj +1 more source
Late‐onset argininosuccinic aciduria in a 72‐year‐old man presenting with fatal hyperammonemia
JIMD Reports, 2021 Argininosuccinate lyase deficiency (ASLD, MIM #207900) is an inherited urea cycle disorder. There are mainly two clinical forms, an acute neonatal form which manifests as life‐threatening hyperammonemia, and a late‐onset form characterised by polymorphic
Laurent Leuger +8 more
doaj +1 more source
Clinical and genetic analysis of five Chinese patients with urea cycle disorders
Molecular Genetics & Genomic Medicine, 2020 Background The urea cycle plays a key role in preventing the accumulation of toxic nitrogenous waste products, including two essential enzymes: ornithine transcarbamylase (OTC) and argininosuccinate lyase (ASL).
Zhenzhu Zheng +6 more
doaj +1 more source
Physical, cognitive, and social status of patients with urea cycle disorders in Japan
Molecular Genetics and Metabolism Reports, 2021 Urea cycle disorders (UCDs) are inherited metabolic diseases that lead to hyperammonemia. Severe hyperammonemia adversely affects the brain. Therefore, we conducted a nationwide study between January 2000 and March 2018 to understand the present status ...
Jun Kido +10 more
doaj +1 more source
Refeeding syndrome in a young woman with argininosuccinate lyase deficiency
Molecular Genetics and Metabolism Reports, 2015 A severely chronically protein and calorie restricted young woman with argininosuccinate lyase deficiency developed transient refeeding syndrome (RFS) and hyperammonemia after modest diet liberalization following initiation of glycerol phenylbutyrate ...
M. Stuy +3 more
doaj +1 more source
Earwax: A potentially useful medium to identify inborn errors of metabolism?
JIMD Reports, 2020 Earwax was investigated as a source to identify patients' different inborn errors of metabolism (IEMs). Acylcarnitines, amino acids, and guanidino metabolites were measured from 28 treated patients with 11 different metabolic disorders including 3 ...
Stefan Krywawych +3 more
doaj +1 more source