Exact Mass: 290.1231
Exact Mass Matches: 290.1231
Found 68 metabolites which its exact mass value is equals to given mass value 290.1231,
within given mass tolerance error 0.001 dalton. Try search metabolite list with more accurate mass tolerance error
0.0002 dalton.
Argininosuccinic acid disodium
Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the argininosuccinate lyase enzyme can lead to argininosuccinate lyase deficiency, which is an inborn error of metabolism. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (argininosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema. Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema. [HMDB] KEIO_ID A039; [MS2] KO008844 KEIO_ID A039
N2-(3-Carboxy-2-hydroxy-1-oxopropyl)arginine
N2-(3-Carboxy-2-hydroxy-1-oxopropyl)arginine is found in pomes. N2-(3-Carboxy-2-hydroxy-1-oxopropyl)arginine is a constituent of the seeds of Vicia faba and the shoots of apple and pear trees. Constituent of the seeds of Vicia faba and the shoots of apple and pear trees. N2-(3-Carboxy-2-hydroxy-1-oxopropyl)arginine is found in pomes and pulses.
N2-(3-Hydroxysuccinoyl)arginine
N2-(3-Hydroxysuccinoyl)arginine is found in pomes. N2-(3-Hydroxysuccinoyl)arginine is a constituent of the shoots of apple and pear trees. Constituent of the shoots of apple and pear trees. N2-(3-Hydroxysuccinoyl)arginine is found in pomes.
Argininosuccinic acid
MS2 deconvoluted using MS2Dec from all ion fragmentation data, MetaboLights identifier MTBLS1040; KDZOASGQNOPSCU_STSL_0151_Argininosuccinic acid_8000fmol_180506_S2_LC02_MS02_235; Spectrum acquired as described in Naz et al 2017 PMID 28641411. Preparation and submission to MassBank of North America by Chaleckis R. and Tada I.
