Year 2020, Volume 8 , Issue 2, Pages 234 - 270 2020-08-23

RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION
İdrar Örneklerinde L-2-Hidroksiglutarik Asitin Kapiler Elektroforez-İndirekt UV Dedeksiyon ile Hızlı Tayini

Mehmet Şerif CANSEVER [1] , Zeynep KALAYCIOĞLU [2] , Tanyel ZÜBARİOĞLU [3] , Ertuğrul KIYKIM [4] , Ayşe AKTUĞLU [5] , Bedia BERKER [6]


Aim: L-2-hydroxyglutaric aciduria (L2HGA) which is autosomal recessive and characterized by psychomotor retardation, cerebellar ataxia, variable macrocephaly, and epilepsy is a rarely seen neurometabolic disease. The disease is biochemically identified by slightly increased L-2HGA levels in urine, cerebrospinal fluid, and plasma. A sensitive and rapid capillary electrophoretic technique was used for the determination of L2HGA in urine. Materials and Method: Since the L2HGA lacks of chromophore, an indirect UV detection method was applied. The running electrolyte consisted of 10 mmol/L 2,6-pyridinedicarboxylic acid (PDC) at pH 5.6 containing 0.1 mmol/L of cetyltrimethylammonium bromide (CTAB). PDC was chosen because of its good chromophoric property for indirect UV detection. CTAB was used as electro-osmotic flow (EOF) reversal. Results: The method was well-validated in the selected conditions. The detection limit of the method L2HGA was 1.16 μmol/L. The migration time of L2HGA was less than 2.0 min. The developed method was applied to the urine samples collected from 16 patients who suffer from L-2-hydroxyglutaric aciduria and 4 healthy volunteers. Conclusion: L-2-hydoxyglutaric acid concentration was found between 48.9 – 1,15 µg/mL in patients.
Amaç: L-2 hidroksiglutarik asidüri (L2HGA) otozomal resesif geçişli, psikomotor gerilik, serebellar ataksi ve değişken makrosefali veya epilepsi ile karakterize edilen nadir görülen nörömetabolik bir hastalıktır. Hastalık biyokimyasal olarak idrarda, beyin omurilik sıvısında ve çok az miktarda plazmada artmış L-2HGA seviyeleri ile tanımlanır. Bu çalışmada, idrarda L-2-hidroksiglutarik asitin tayini için hızlı ve hassas bir kapiler elektroforez yöntemi geliştirilmiştir. Materyal ve Metot: L-2-hidroksiglutarik asit kromofor gruba sahip olmadığı için, indirekt UV deteksiyon metodu uygulanmıştır. Çalışma tamponu pH 5.6’da 0.1 mmol/L setiltrimetilamonyum bromür (CTAB) içeren 10 mmol/L 2,6-piridindikarboksilik asitten (PDC) oluşmaktadır. PDC iyi bir kromoforik özellik göstermesinden dolayı indirekt deteksiyon için uygundur. CTAB elektro-osmotik akışı (EOF) ters çevirmek için kullanılmıştır. Bulgular: L-2-hidroksiglutarik asit için tayin limiti 1.16 μmol/L’dir. Geliş zamanı ise 2.0 dakikadan daha az bir süredir. Geliştirilen metod 16 L-2-hidroksiglutarik asidüri hastasından ve 4 sağlıklı gönüllüden toplanan idrar örneklerine uygulanmıştır. Sonuç: Hasta bireylerin idrarındaki L-2-hidroksiglutarik asitin konsantrasyonu 48.9 – 1,15 µg/mL arasında bulunmuştur.
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Primary Language en
Subjects Medicine
Journal Section Orginal Article
Authors

Orcid: 0000-0003-3315-5124
Author: Mehmet Şerif CANSEVER (Primary Author)
Institution: İSTANBUL ÜNİVERSİTESİ- CERRAHPAŞA
Country: Turkey


Orcid: 0000-0002-0967-0997
Author: Zeynep KALAYCIOĞLU
Institution: İSTANBUL TEKNİK ÜNİVERSİTESİ
Country: Turkey


Orcid: 0000-0002-7159-4008
Author: Tanyel ZÜBARİOĞLU
Institution: İSTANBUL ŞİŞLİ HAMİDİYE ETFAL SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ
Country: Turkey


Orcid: 0000-0001-5170-5594
Author: Ertuğrul KIYKIM
Institution: CERRAHPAŞA TIP PR.
Country: Turkey


Orcid: 0000-0001-7256-0750
Author: Ayşe AKTUĞLU
Institution: CERRAHPAŞA TIP PR.
Country: Turkey


Orcid: 0000-0001-9406-6681
Author: Bedia BERKER
Institution: İSTANBUL TEKNİK ÜNİVERSİTESİ
Country: Turkey


Dates

Publication Date : August 23, 2020

Bibtex @research article { nkmj687468, journal = {Namık Kemal Tıp Dergisi}, issn = {}, eissn = {2587-0262}, address = {Namık Kemal Üniversitesi Tıp Fakültesi Dekanlığı, Namık Kemal Mahallesi Kampüs Caddesi No:1 59100-Süleymanpaşa-TEKİRDAĞ}, publisher = {Namik Kemal University}, year = {2020}, volume = {8}, pages = {234 - 270}, doi = {10.37696/nkmj.687468}, title = {RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION}, key = {cite}, author = {Cansever, Mehmet Şerif and Kalaycıoğlu, Zeynep and Zübarioğlu, Tanyel and Kıykım, Ertuğrul and Aktuğlu, Ayşe and Berker, Bedia} }
APA Cansever, M , Kalaycıoğlu, Z , Zübarioğlu, T , Kıykım, E , Aktuğlu, A , Berker, B . (2020). RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION . Namık Kemal Tıp Dergisi , 8 (2) , 234-270 . DOI: 10.37696/nkmj.687468
MLA Cansever, M , Kalaycıoğlu, Z , Zübarioğlu, T , Kıykım, E , Aktuğlu, A , Berker, B . "RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION" . Namık Kemal Tıp Dergisi 8 (2020 ): 234-270 <https://dergipark.org.tr/en/pub/nkmj/issue/56143/687468>
Chicago Cansever, M , Kalaycıoğlu, Z , Zübarioğlu, T , Kıykım, E , Aktuğlu, A , Berker, B . "RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION". Namık Kemal Tıp Dergisi 8 (2020 ): 234-270
RIS TY - JOUR T1 - RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION AU - Mehmet Şerif Cansever , Zeynep Kalaycıoğlu , Tanyel Zübarioğlu , Ertuğrul Kıykım , Ayşe Aktuğlu , Bedia Berker Y1 - 2020 PY - 2020 N1 - doi: 10.37696/nkmj.687468 DO - 10.37696/nkmj.687468 T2 - Namık Kemal Tıp Dergisi JF - Journal JO - JOR SP - 234 EP - 270 VL - 8 IS - 2 SN - -2587-0262 M3 - doi: 10.37696/nkmj.687468 UR - https://doi.org/10.37696/nkmj.687468 Y2 - 2020 ER -
EndNote %0 Namık Kemal Tıp Dergisi RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION %A Mehmet Şerif Cansever , Zeynep Kalaycıoğlu , Tanyel Zübarioğlu , Ertuğrul Kıykım , Ayşe Aktuğlu , Bedia Berker %T RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION %D 2020 %J Namık Kemal Tıp Dergisi %P -2587-0262 %V 8 %N 2 %R doi: 10.37696/nkmj.687468 %U 10.37696/nkmj.687468
ISNAD Cansever, Mehmet Şerif , Kalaycıoğlu, Zeynep , Zübarioğlu, Tanyel , Kıykım, Ertuğrul , Aktuğlu, Ayşe , Berker, Bedia . "RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION". Namık Kemal Tıp Dergisi 8 / 2 (August 2020): 234-270 . https://doi.org/10.37696/nkmj.687468
AMA Cansever M , Kalaycıoğlu Z , Zübarioğlu T , Kıykım E , Aktuğlu A , Berker B . RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION. NKMJ. 2020; 8(2): 234-270.
Vancouver Cansever M , Kalaycıoğlu Z , Zübarioğlu T , Kıykım E , Aktuğlu A , Berker B . RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION. Namık Kemal Tıp Dergisi. 2020; 8(2): 234-270.
IEEE M. Cansever , Z. Kalaycıoğlu , T. Zübarioğlu , E. Kıykım , A. Aktuğlu and B. Berker , "RAPID DETERMINATION OF L-2-HYDROXYGLUTARIC ACID IN URINE SAMPLES BY CAPILLARY ELECTROPHORESIS WITH INDIRECT UV DETECTION", Namık Kemal Tıp Dergisi, vol. 8, no. 2, pp. 234-270, Aug. 2020, doi:10.37696/nkmj.687468