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Cystinuria is a genetic disorder, and in severe cases, it might lead to kidney failure. As an important biomarker for cystinuria, the level of arginine (Arg) in urine is a vital indicator for cystinuria screening. Therefore, it is urgently needed to detect Arg with high selectivity and sensitivity. In this work, a boric acid functionalized Zr-based metal-organic framework UiO-PhbA is prepared by grafting phenylboronic acid on UiO-66-NH through a Schiff base reaction using a covalent post-synthesis modification (CPSM) strategy. The prepared UiO-PhbA exhibits a sensitive and specific fluorescence "turn-on" response to Arg and can be exploited to detect Arg in human serum and urine samples with a broad linear range of 0.6-350 µM and low limit of detection (LOD) of 18.45 nM. This study provides a new and reliable rapid screening protocol for sulfite oxidase deficiency-related diseases.
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http://dx.doi.org/10.1007/s00604-024-06519-6 | DOI Listing |
J Nephrol
January 2025
Section of Nephrology, Department of Medicine, Università Degli Studi Di Verona, Verona, Italy.
Background: Cystinuria is a rare genetic disease characterized by impaired tubular transport of cystine. Clinical features of cystinuria mainly include nephrolithiasis and its complications, although cystinuric patients may present with other comorbidities. There are currently no data on bone features of patients with cystinuria.
View Article and Find Full Text PDFMikrochim Acta
June 2024
Key Laboratory of Eco-Functional Polymer Materials of the Ministry of Education, Key Lab of Bioelectrochemistry and Environmental Analysis of Gansu Province, College of Chemistry and Chemical Engineering, Northwest Normal University, Gansu International Scientific and Technological Cooperation Base
Cystinuria is a genetic disorder, and in severe cases, it might lead to kidney failure. As an important biomarker for cystinuria, the level of arginine (Arg) in urine is a vital indicator for cystinuria screening. Therefore, it is urgently needed to detect Arg with high selectivity and sensitivity.
View Article and Find Full Text PDFOrphanet J Rare Dis
May 2024
Diagnosis and Treatment of Congenital Metabolic Diseases, University Clinical Hospital of Santiago de Compostela, A Coruña, Spain.
Background: There is a notable lack of harmonisation in newborn screening (NBS) programmes worldwide. The Galician programme for early detection of inborn errors of metabolism (IEM) was one of the first NBS programmes in Europe to incorporate mass spectrometry (July 2000). This programme currently screens for 26 IEMs in dried blood and urine samples collected 24-72 h after birth.
View Article and Find Full Text PDFTalanta
September 2023
Key Lab of Eco-Environments Related Polymer Materials of MOE; Key Lab of Bioelectrochemistry and Environmental Analysis of Gansu Province; College of Chemistry and Chemical Engineering, Northwest Normal University, Lanzhou, 730070, PR China. Electronic address:
Arginine (Arg) is considered a valuable biomarker for various diseases, including cystinuria, and its concentration level holds significant implications for human health. To achieve the purposes of food evaluation and clinical diagnosis, it is imperative to develop a rapid and facile method for selective and sensitive determination of Arg. In this work, a novel fluorescent material (Ag/Eu/CDs@UiO-66) was synthesized by encapsulating carbon dots (CDs), Eu and Ag into UiO-66.
View Article and Find Full Text PDFAnal Chem
July 2022
Analytical & Testing Center, Sichuan University, Chengdu, Sichuan 610064, China.
Arginine is considered as a biomarker of cystinuria and other diseases, and thus, it is of urgency to develop a simple and rapid method with high sensitivity and selectivity for arginine detection to meet the demand of on-site analysis and bedside diagnosis. In this work, a lanthanide metal-organic framework, La(TATB), was prepared using a triazine-based planar ligand, 4,4',4″--triazine-2,4,6-triyltribenzoate (HTATB), and lanthanide ion (La). La(TATB) can be used as a highly photosensitive agent to activate molecular oxygen to O to achieve efficient photosensitive oxidation of arginine accompanied by strong blue fluorescence emission under 302 nm UV irradiation.
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