Determination of NTBC in serum samples from patients with hereditary tyrosinemia type I by capillary electrophoresis.
Hereditary tyrosinemia type I is a serious metabolic disorder leading to liver failure. 2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) is a relatively new drug which is used to prevent the accumulation of toxic metabolites in patients with hereditary tyrosinemia type I. In the present study, we have developed a new, simple, fast, and cost-effective capillary electrophoresis method for the quantitative monitoring of this drug in serum samples. Micellar electrochromatographic separation of NTBC was performed using 20 mmol/L phosphate and 40 mmol/L sodium dodecylsulfate (SDS) at pH 12 as running electrolyte. Separation of NTBC was achieved in around 4 min. Reproducibilities of migration times and corrected peak areas of NTBC (as R.S.D.%) were found as 0.73 and 1.99, respectively. The detection limit was 3.17 and the quantification limit was 10.6 micromol/L for NTBC using UV detection at 278 nm. The utility of the method was demonstrated by the detection of NTBC in serum samples from patients with hereditary tyrosinemia type I using this drug.
Department of Pediatrics, Division of Metabolism, Cerrahpaşa Medical School, University of Istanbul, Cerrahpaşa 3430, Istanbul, Turkey.
M Serif
MS
Cansever
A Ciğdem
AC
Aktuğlu-Zeybek
F Bedia
FB
Erim
D003512:Cyclohexanones / Q000097:blood*; D019075:Electrophoresis, Capillary / Q000191:economics / Q000379:methods*; D004791:Enzyme Inhibitors / Q000097:blood*; D006801:Humans; D057230:Limit of Detection; D009579:Nitrobenzoates / Q000097:blood*; D013997:Time Factors; D020176:Tyrosinemias / Q000188:drug therapy*
D003512:Cyclohexanones; D004791:Enzyme Inhibitors; D009579:Nitrobenzoates; C077073:nitisinone