Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH₄)-responsiveness in patients with hyperphenylalaninemia.

ABSTRACT: Blood phenylalanine-to-tyrosine (Phe/Tyr) ratio is an important indicator of metabolic control in phenylketonuria patients. We present the data that highlights the role of Phe/Tyr-ratio in the evaluation of tetrahydrobiopterin (BH₄)-responsiveness in patients with hyperphenylalaninemia. Our data complements the results from the original research article by Tansek et al., 2012 [1]. We performed a BH₄-loading test in 32 patients after four days of increased protein intake (2000 mg/kg body weight). Blood sampling was performed 96, 72, 48, 24, 16 h, and moments before oral administration of BH₄ in a dose of 20 mg/kg body weight. Additional blood samples were collected 8 and 24 h after its administration. Phenylalanine (Phe) and Tyrosine (Tyr) levels were determ

SUBMITTER: Lampret BR 

PROVIDER: S-EPMC8841992 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature