Tetrahydrobiopterin in phenylketonuria: Who can benefit? [PDF]
, 2020 Phenylketonuria, abbreviated PKU, is a rare inherited metabolic disease. In this disease, a building block of protein (an amino acid) called phenylalanine cannot be converted to tyrosine. This results in high phenylalanine concentrations in blood and brain. If left untreated, this especially results in severe developmental delay as well as epilepsy and
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Food Science &Nutrition, Volume 13, Issue 12, December 2025.BPA exposure in rats and HUVECs impaired aortic contractility, increased lipid peroxidation, ROS, NO, VCAM‑1, and p‑eNOS, and blunted relaxant responses. Co‑treatment with NSO or TQ restored KCl/PE‑induced contractions, enhanced ACh‑ and SNP‑mediated vasorelaxation, and lowered oxidative/nitrosative stress and adhesion molecule expression.
Masoumeh Fadishei +6 more
wiley +1 more source
The key role of nitric oxide in hypoxia: hypoxic vasodilation and energy supply-demand matching [PDF]
, 2013 Significance: a mismatch between energy supply and demand induces tissue hypoxia with the potential to cause cell death and organ failure. Whenever arterial oxygen concentration is reduced, increases in blood flow - 'hypoxic vasodilation' - occur in an ...
Alex Dyson +23 more
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Dysregulated Choline, Methionine, and Aromatic Amino Acid Metabolism in Patients with Wilson Disease: Exploratory Metabolomic Profiling and Implications for Hepatic and Neurologic Phenotypes. [PDF]
, 2019 Wilson disease (WD) is a genetic copper overload condition characterized by hepatic and neuropsychiatric symptoms with a not well-understood pathogenesis.
Czlonkowska, Anna +6 more
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Living with phenylketonuria in adulthood: the PKU ATTITUDE study [PDF]
, 2018 Dietary treatment is the cornerstone of therapy for phenylketonuria (PKU), but adherence to low- phenylalanine diet progressively decreases after adolescence.
Bensi, Giulia +11 more
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Tetrahydrobiopterin protects soluble guanylate cyclase against oxidative inactivation
Pteridines, 2013 Tetrahydrobiopterin (BH4) is a major endogenous vasoprotective agent that improves endothelial function by increasing nitric oxide (NO) synthesis and scavenging of superoxide and peroxynitrite.
Schmidt Kurt +2 more
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Molecular diagnosis of phenylketonuria: From defective protein to disease-causing gene mutation [PDF]
Journal of Medical Biochemistry, 2009 Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism, with an average incidence of 1/10000 in Caucasians. PKU is caused by more than 500 mutations in the phenylalanine hydroxylase gene (PAH) which result in phenylalanine ...
Pavlović Sonja, Stojiljković Maja
doaj
Pteridines, 1995 Incorporation of fatty acids into phospholipids has been investigated using samples of rat live tissue homogenate, Krebs-Ringer-phosphate buffer (pH = 7A) containing 0.3% albumin, farry acid mixture and glycerol.
Rudzite Vera +4 more
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Compensatory Feto-Placental Upregulation of the Nitric Oxide System during Fetal Growth Restriction [PDF]
, 2012 Background: Fetal Growth Restriction is often associated with a feto-placental vascular dysfunction conceivably involving endothelial cells. Our study aimed to verify this pathogenic role for feto-placental endothelial cells and, coincidentally ...
Begliuomini, Silvia +11 more
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Tetrahydrobiopterin, superoxide, and vascular dysfunction [PDF]
Free Radical Biology and Medicine, 2009 (6R)-5,6,7,8-Tetrahydrobiopterin (BH(4)) is an endogenously produced pterin that is found widely distributed in mammalian tissues. BH(4) works as a cofactor of aromatic amino acid hydroxylases and nitric oxide synthases. In the vasculature a deficit of BH(4) is implicated in the mechanisms of several diseases including atherosclerosis, hypertension ...
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