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Tetrahydrobiopterin (BH 4, THB), also known as sapropterin (), [5] [6] is a cofactor of the three aromatic amino acid hydroxylase enzymes, [7] used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline), and is a cofactor for the ...
Tetrahydrobiopterin deficiency (THBD, BH 4 D) is a rare metabolic disorder that increases the blood levels of phenylalanine.Phenylalanine is an amino acid obtained normally through the diet, but can be harmful if excess levels build up, causing intellectual disability and other serious health problems.
BH4 is the principal active cofactor. BH4 synthesis occurs through two principal pathways; the de novo pathway involves three enzymatic steps and proceeds from GTP, while the salvage pathway converts sepiapterin to BH4 using dihydrofolate reductase. [4] In addition, BH2 is recycled to BH4 by dihydrobiopterin reductase.
Ball-and-stick model of the tetrahydroborate anion, [BH 4] −. Borohydride refers to the anion [B H 4] −, which is also called tetrahydridoborate, and its salts. [1] Borohydride or hydroborate is also the term used for compounds containing [BH 4−n X n] −, where n is an integer from 0 to 3, for example cyanoborohydride or cyanotrihydroborate [BH 3 (CN)] − and triethylborohydride or ...
The current version of ShuntCheck was developed in 2011-2012 funded by grants from the National Institute of Health [17] [18] [19] and was cleared by the US FDA in 2013. [1] ...
Each of the types of shunts listed above can be composed of a tube or catheter and various types of valves, although they can just be composed of the tubing or catheter. Below is a list of valves that are used in lumbar–peritoneal shunts (LP shunts) and Cerebral shunts (for a more detailed list of the types of valves see type of valves): Delta
A Peritoneovenous shunt: (also called Denver shunt) [2] is a shunt which drains peritoneal fluid from the peritoneum into veins, usually the internal jugular vein or the superior vena cava. It is sometimes used in patients with refractory ascites. It is a long tube with a non-return valve running subcutaneously from the peritoneum to the ...
The incidence of univentricular heart malformations is estimated at 0.1 to 0.4 per 1,000 live births. [3] In the neonatal period, these patients depend on an aortopulmonary shunt that is maintained medically with prostaglandin and then surgically with an initial cardiac shunt procedure.