TY - JOUR TI - RAPID DETERMINATION OF L-2-HYDOXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION AB - Aim: L-2-hydroxyglutaric aciduria (L2HGA) which is autosomal recessive and characterized by psychomotor retardation, cerebellarataxia, variable macrocephaly, and epilepsy is a rarely seen neurometabolic disease. The disease is biochemically identified by slightlyincreased L-2HGA levels in urine, cerebrospinal fluid, and plasma.Materials and Methods: A sensitive and rapid capillary electrophoretic technique was used for the determination of L2HGA in urine.Since the L2HGA lacks of chromophore, an indirect UV detection method was applied.Results: The running electrolyte consisted of 10 mmol/L 2,6-pyridinedicarboxylic acid (PDC) at pH 5.6 containing 0.1 mmol/L ofcetyltrimethylammonium bromide (CTAB). PDC was chosen because of its good chromophoric property for indirect UV detection. CTABwas used as electro-osmotic flow (EOF) reversal. The method was well-validated in the selected conditions. The detection limit of themethod L2HGA was 1.16 μmol/L. The migration time of L2HGA was less than 2.0 min.Conclusion: The developed method was applied to the urine samples collected from 16 patients who suffer from L-2-hydroxyglutaricaciduria and 4 healthy volunteers. AU - KIYKIM, Ertuğrul AU - ZÜBARİOĞLU, Tanyel AU - Kalaycıoğlu, Zeynep AU - ERİM, Fatma Bedia AU - ZEYBEK, Ayşe Çiğdem AKTUĞLU AU - CANSEVER, Mehmet Şerif DO - 10.37696/nkmj.687468 PY - 2020 JO - Namık Kemal Tıp Dergisi VL - 8 IS - 2 SN - 2587-0262 SP - 264 EP - 270 DB - TRDizin UR - http://search/yayin/detay/460118 ER -