Results 91 to 100 of about 14,507 (223)

Tetrahydrobiopterin, its mode of action on phenylalanine hydroxylase, and importance of genotypes for pharmacological therapy of phenylketonuria

, 2013
In about 20%-30% of phenylketonuria (PKU) patients (all phenotypes of PAH deficiency), Phe levels may be controlled through phenylalanine hydroxylase cofactor tetrahydrobiopterin therapy.
Blau, Nenad, Heintz, Caroline, Cotton, Richard G H, Heintz C, Blau N, Cotton RG   +5 more
core   +1 more source

Tetrahydrobiopterin precursor sepiapterin provides protection against neurotoxicity of 1-methyl-4-phenylpyridinium in nigral slice cultures

, 2003
Udgivelsesdato: 2003-AprComplex-I inhibition and oxidative processes have been implicated in the loss of nigral dopamine neurones in Parkinson's disease and the toxicity of MPTP and its metabolite MPP+.
Gramsbergen, Jan Bert, Morten Meyer, Zimmer, Jens, Meyer, Morten, Jakob Torp Madsen, Christian Hesslinger, Jens Zimmer, Madsen, Jakob Torp, Jan Bert Gramsbergen, Hesslinger, Christian, Pernille Jansen, Jansen, Pernille   +11 more
core   +1 more source

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, Werner Ernst R., Mayer Bernd   +2 more
doaj   +1 more source

[Tetrahydrobiopterin as putative mediator in septic shock.]

, 2013
Enhanced serum levels neopterin are associated with various immune-related diseases like septic shock and vascular leakage syndrome. Tetrahydrobiopterin (THB, 100 muM) but not bioterin induces appreciable endothelium-dependent vasodilatation and enhances
Wemer J, Eigeman L, te Biesebeek JD, van Amsterdam JGC, van de Kuil A   +4 more
core  

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  

Effect of Sepiapterin, 7,8-Dihydrobiopterin, 5,6,7,8-Tetrahydrobiopterin and Xanthopterin on Cholesterol and Phospholipid Content and Phospholipid Biosynthesis in vitro

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, Jurika Edite, Baier-Bitterlich Gabriele, Wachter Helmut, Fuchs Dietmar   +4 more
doaj   +1 more source

Say NO to ROS: Their Roles in Embryonic Heart Development and Pathogenesis of Congenital Heart Defects in Maternal Diabetes

Antioxidants, 2019
Congenital heart defects (CHDs) are the most prevalent and serious birth defect, occurring in 1% of all live births. Pregestational maternal diabetes is a known risk factor for the development of CHDs, elevating the risk in the child by more than four ...
Anish Engineer, Tana Saiyin, Elizabeth R. Greco, Qingping Feng   +3 more
doaj   +1 more source

Regulation of melanin biosynthesis in the human epidermis by tetrahydrobiopterin.

, 1994
The participation of (6R) 5,6,7,8-tetrahydrobiopterin (6-BH4) in regulating the tyrosine supply for melanin biosynthesis was investigated by the examination of human keratinocytes, melanocytes, and epidermal suction blisters from normal human skin and ...
Gütlich, M., Lemke, K.R., Hitzemann, K., Schallreuter, K.U., Ziegler, I., Swanson, N.N., Wood, J.M., Pittelkow, M.R., Rödl, W.   +8 more
core   +1 more source

Native and Phosphorylated Bovine Adrenal Tyrosine 3-Monooxygenase. Interactions with Tetrahydropterins and Substrate and Stability of the Formed 4a-Hydroxy-Tetrahydrobiopterin

Pteridines, 1989
The catalytic activity of tyrosine 3-monooxygenase (tyrosine hydroxylase) is dependent on a tetrahydropterin cofactor and ezyme-bound iron. The oxidation of tetrahydrobiopterin by bovine adrenal tyrosine hydroxylase was studied by high performance liquid
Haavik Jan, Andersson Kristoffer K., Flatmark Torgeir, Petersson Leif   +3 more
doaj   +1 more source

Pterin Metabolism In Haemodialysis Patients

Pteridines, 1989
Patients undertaking maintenance haemodialysis may develop a variety of neurological disorders, the most serious of which is aluminium induced encephalopathy.
Altmann Paul, Sawyer Nick, Cunningham John, Marsh Frank, Hamon Chris, Blair John A.   +5 more
doaj   +1 more source