2020
DOI: 10.18388/abp.2020_5164
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Determination of amino acid profile for argininosuccinic aciduria disorder using High-Performance Liquid Chromatography with fluorescence detection

Abstract: Argininosuccinic aciduria is an autosomal, recessive amino acid disorder that is caused by a deficiency of the argininosuccinate lyase enzyme. Citrulline is the most significant marker to detect this disorder. We used the High-performance liquid chromatography with fluorescence detection with 450 nm emission and 330 nm excitation wavelengths, 15 mmol/L potassium dihydrogen phosphate and 5 mmol/L dipotassium hydrogen phosphate as Mobile Phase A, and 50 mL water, 250 mL acetonitrile, and 200 mL methanol as Mobil… Show more

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Cited by 3 publications
(3 citation statements)
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“…Metabolic acidosis is also one of the reasons that cause RF. 32 Argininosuccinic acid is a precursor of arginine produced in the citric acid cycle by argininosuccinate lyase (ASA), 33 and increased excretion of ASA increases the risk of hyperammonemia. 34 Both N -acetyl- l -aspartic acid and argininosuccinic acid are aspartic acid derivatives, and the changes in their contents can reflect the glomerular filtration function.…”
Section: Discussionmentioning
confidence: 99%
“…Metabolic acidosis is also one of the reasons that cause RF. 32 Argininosuccinic acid is a precursor of arginine produced in the citric acid cycle by argininosuccinate lyase (ASA), 33 and increased excretion of ASA increases the risk of hyperammonemia. 34 Both N -acetyl- l -aspartic acid and argininosuccinic acid are aspartic acid derivatives, and the changes in their contents can reflect the glomerular filtration function.…”
Section: Discussionmentioning
confidence: 99%
“…The resulting mixture was injected into HPLC after 2 min at room temperature. Reverse phase HPLC (RP-HPLC) was done on an Azura Knauer pump (Germany) and a fluorescence detector (Shimadzu Prominence RF-20 A, Japan) in 350 nm excitation/450 nm emission wavelength criteria by gradient elution on a C18 column (150 * 4.6 mm, 5 μm, Macherey-Nagel, Germany), using Mobile-phase A (1 g K2PO4 + 0.5 g KH2PO4 in 500 mL distilled water), and mobile- phase B (100 mL distilled water, 150 mL acetonitrile, and 300 mL isopropanol) [ 41 , 42 ]. Gradient elution was linear from 10 to 100% B at a flow rate of 1.2 mL/min in 35 min.…”
Section: Methodsmentioning
confidence: 99%
“…Mobile phase B: 75 mL ultrapure water, 225 mL acetonitrile, and 200 mL methanol. The flow rate and column oven temperature were 1.3 mL/min and 30 °C (Salmanizadeh and Sahi 2020). Vinblastine, vincristine, tryptophan standards, methanol, ammonia, diethyl ether, acetonitrile, methoxy ethanol, and ultrapure water were purchased from Sigma Aldrich (Burlington, USA).…”
Section: Methodsmentioning
confidence: 99%