BH4 tetrahydrobiopterin

Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies. (Mol Genet Metab, 2011) [PMID:21937252]
Delivery of exogenous tetrahydrobiopterin (BH4) to cells of target organs: role of salvage pathway and uptake of its precursor in effective elevation of tissue BH4. (Mol Genet Metab, 2005) [PMID:16256391]
Sepiapterin for the treatment of phenylketonuria. (Expert Opin Pharmacother, 2025) [PMID:40272408]

_Worker-drafted node — pending editorial review._

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Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies. (2011) pubmed
Delivery of exogenous tetrahydrobiopterin (BH4) to cells of target organs: role of salvage pathway and uptake of its precursor in effective elevation of tissue BH4. (2005) pubmed
Sepiapterin for the treatment of phenylketonuria. (2025) pubmed
Disruption of tetrahydrobiopterin (BH4) biosynthesis pathway affects cuticle pigmentation in Henosepilachna vigintioctopunctata. (2023) pubmed
Catecholamines and Parkinson's disease: tyrosine hydroxylase (TH) over tetrahydrobiopterin (BH4) and GTP cyclohydrolase I (GCH1) to cytokines, neuromelanin, and gene therapy: a historical overview. (2024) pubmed
Defining tetrahydrobiopterin (BH4)-responsiveness in PKU. (2008) pubmed
Tetrahydrobiopterin (BH4) in septic shock: a key to solving the nitric oxide puzzle and opening the microcirculation? (2012) pubmed
Tyrosine hydroxylase (TH), its cofactor tetrahydrobiopterin (BH4), other catecholamine-related enzymes, and their human genes in relation to the drug and gene therapies of Parkinson's disease (PD): historical overview and future prospects. (2016) pubmed
Role of endothelial and macrophage tetrahydrobiopterin in development and progression of atherosclerosis: BH4 puzzle solved? (2018) pubmed
Tetrahydrobiopterin (BH4) responsiveness in neonates with hyperphenylalaninemia: a semi-mechanistically-based, nonlinear mixed-effect modeling. (2015) pubmed