Defining tetrahydrobiopterin (BH4)-responsiveness in PKU

Defining tetrahydrobiopterin (BH₄)-responsiveness in PKU

This is a preview of subscription content, log in via an institution to check access.

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Burnett JR (2007) Sapropterin dihydrochloride (Kuvan/Phenoptin), an orally active synthetic form of BH₄ for the treatment of phenylketonuria. IDrugs 10(11): 805–813.
PubMed CAS Google Scholar
Fiege B, Blau N (2007) Assessment of tetrahydrobiopterin (BH₄)-responsiveness in phenylketonuria. J Pediatr 150: 627–630.
Article PubMed CAS Google Scholar
Levy L, Milanowski A, Chakrapani A, et al (2007) Efficacy of sapropterin dihydrochloride (tetrahydrobiopterin, 6R-BH₄) for reduction of phenylalanine concentration in patients with phenylketonuria: a phase III randomized placebo-controlled study. Lancet 370: 504–510.
Article PubMed CAS Google Scholar
Zurflüh MR, Zschocke J, Lindner M, et al (2008) Molecular genetics of tetrahydrobiopterin responsive phenylalanine hydroxylase deficiency. Hum Mutat 29(1): 167–175
Article PubMed Google Scholar

Division of Clinical Chemistry and Biochemistry, University Children’s Hospital, Steinwiesstrasse 75, CH-8032, Zürich, Switzerland
Nenad Blau

Authors

Nenad Blau
View author publications
You can also search for this author in PubMed Google Scholar

Correspondence to Nenad Blau.

Blau, N. Defining tetrahydrobiopterin (BH₄)-responsiveness in PKU. J Inherit Metab Dis 31, 2–3 (2008). https://doi.org/10.1007/s10545-007-9979-1

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.