What is BH4 supplement?
What is BH4 supplement?
BH4 Assist is an additional supplement choice for TRIAD 2 BRAIN/DEPRESSION support. BH4 plays a critical role in both neurotransmitter and nitric oxide production. It can be depleted by genetic polymorphisms in MTHFR A1298C, DHFR, high levels of ammonia (from CBS mutations), oxidative stress, or lack of SAMe or NADH.
What is the importance of Biopterin?
Biopterin is an essential cofactor in hydroxylase enzymes and is necessary for the synthesis of serotonin. NADH and NADPH are involved in nearly every redox reaction in all living cells.
What is active Biopterin?
BH4 is the principal active cofactor, and a recycling pathway converts BH2 to BH4.
Are there disorders of the metabolism of biopterin?
Disorders of biopterin metabolism Defects in the metabolism or regeneration of tetrahydrobiopterin (BH4) were initially discovered in patients with hyperphenylalaninaemia who had progressive neurological deterioration despite optimal metabolic control (malignant hyperphenylalaninaemia). BH4 is an essential cofactor not only for phen …
How are the eyes affected by tetrahydrobiopterin deficiency?
Some affected individuals may develop abnormal movements of the eyes that can range from brief upward rolling of the eyes to oculogyric crises, in which the eyes roll upward for a sustained period of time. Sometimes, the eyes may roll downward or move toward each other (converge).
What happens to biopterin and neopterin in GTPCH deficiency?
In GTPCH deficiency, levels of biopterin and neopterin are abnormally low. In PTPS deficiency, neopterin is highly elevated and biopterin is very low or absent. In DHPR deficiency, biopterin is highly elevated and neopterin is normal or slightly elevated, but in some patients both metabolites can be normal (see below).
Why is tetrahydrobiopterin considered a cofactor in the body?
A cofactor is a non-protein substance in the body that enhances or is necessary for the proper function of certain enzymes. When tetrahydrobiopterin is deficient, the chemical balance within the body is upset.