Related Experiment Video
Updated: Jun 1, 2026

Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay
Published on: February 20, 2015
Blood phenylalanine clearance and BH(4)-responsiveness in classic phenylketonuria
Miroslaw Bik-Multanowski1, Jacek J Pietrzyk
1Department of Medical Genetics, Jagiellonian University, Poland. AgMir@mp.pl
Abstract:
Tetrahydrobiopterin (BH(4)) has been shown to decrease blood phenylalanine concentration in selected patients with phenylketonuria who can be identified with use of the BH(4)-loading test. However, the results of the test could be biased due to hydroxylation-independent blood phenylalanine clearance. Considering of this effect is necessary in patients with classic phenylketonuria, particularly in "slow responders," in whom borderline decrease in blood phenylalanine concentration is typically observed as a result of BH(4)-loading.
Related Concept Videos
Inborn Errors of Metabolism
Pharmacokinetics in Pediatric Patients: Drug Metabolism
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption
Pharmacokinetics in Pediatric Patients: Drug Distribution
Nonlinear Pharmacokinetics: Dependence of Elimination Half-Life and Dose Clearance
A study on guinea pigs examined the...
