Determination of NTBC in serum samples from patients with hereditary tyrosinemia type I by capillary electrophoresis.

Abstract

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.

• Authors: M Serif Cansever, A Ciğdem Aktuğlu-Zeybek, F Bedia Erim
• Journal: Talanta (Talanta) Vol. 80 Issue 5 Pg. 1846-8 (Mar 15 2010) ISSN: 1873-3573 [Electronic] Netherlands
• PMID: 20152421 (Publication Type: Journal Article, Validation Study)
• Copyright: Copyright (c) 2009 Elsevier B.V. All rights reserved.

Chemical References

• Cyclohexanones
• Enzyme Inhibitors
• Nitrobenzoates
• nitisinone

Topics

• Cyclohexanones (blood)
• Electrophoresis, Capillary (economics, methods)
• Enzyme Inhibitors (blood)
• Humans
• Limit of Detection
• Nitrobenzoates (blood)
• Time Factors
• Tyrosinemias (drug therapy)