what is bh4 used for

What is the function of BH4?

BH4, or tetrahydrobiopterin, is used for many imperative and fundamental processes in the body. What Decreases BH4?

What is R1 BH4 used to treat?

R BH4 is being used in clinical trials to treat autism, ADHD, hypertension, endothelial dysfunction, and chronic kidney disease. R 1. BH4 Reverses PKU

What is the best BH4 supplement for me?

Direct BH4 supplementation is the most obvious target in those who are BH4 deficient, however some forum posters report minor adverse effects. In these cases indirect supplementation with MTHF may prove more beneficial in some cases.

What is BH4 and why is it important for autism?

BH4 may improve autistic symptoms such as adaptability, verbal expression, social responsiveness and interactions, communication, cognitive abilities, hyperactivity, and inappropriate speech. Cerebrospinal fluid BH4 levels are 42% lower in children with Autism.

What is the function of BH4?

Tetrahydrobiopterin (BH4) functions as a cofactor for several important enzyme systems, and considerable evidence implicates BH4 as a key regulator of endothelial nitric oxide synthase (eNOS) in the setting of cardiovascular health and disease.

What is BH4 supplement?

BH4 is a naturally occurring essential cofactor of the three aromatic amino acid hydroxlase enzymes used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin, melatonin, dopamine, norepinephrine, epinephrine, and is a cofactor for the production of nitric oxide by ...

What is BH4 made from?

BH4 is made from the molecule GTP (guanosine triphosphate). GTP is converted into BH4 in three stages, which are catalysed (in order) by the enzymes GTPCH, PTPS and SR. These enzymes are coded for, respectively, by the GCH1, PTS and SR genes. Interestingly, rare mutations in these genes can lead to deficiency of BH4.

What is BH4 deficiency?

Tetrahydrobiopterin (BH4) deficiency causes the body to build up an abnormally high level of phenylalanine, one of the building blocks of proteins. In addition, BH4 deficiency leads to low levels of certain neurotransmitters, chemical messengers that control many body functions.

How do I increase BH4?

Rather, studies have shown that MTHF supplementation can increase BH4 levels by preventing its oxidation into BH2 and by supplementing BH4 activity with NOS enzymes further preventing its degradation.

Does Royal Jelly increase BH4?

Royal Jelly has been included for its naturally occurring BH4. Lithium has been noted in clinical research to increase BH4 levels. For those with low levels of neurotransmitters, NO or for more robust support, this product pairs well with SER Accelerator or 5-HTP.

What is the name of BH4?

Borohydride is a boron hydride. A class of inorganic or organic compounds that contain the borohydride (BH4-) anion.

How is BH4 deficiency treated?

Treatment of BH4 deficiencies consists of BH4 supplementation (2-20 mg/kg per day) or diet to control blood phenylalanine concentration and replacement therapy with neurotransmitters precausers (L-dopa/CarbiDOPA and 5-hydroxytryptophan), and supplements of folinic acid in DHPR deficiency.

How do you test for BH4 deficiency?

Molecular genetic testing can confirm a diagnosis of these disorders. Molecular genetic testing can detect mutations in the specific genes known to cause tetrahydrobiopterin deficiency. The test is often expensive and often not necessary to confirm a diagnosis of a disorder of tetrahydrobiopterin deficiency.

Is BH4 an enzyme?

Tetrahydrobiopterin (BH4) is essential for the biosynthesis of dopamine, noradrenaline, and serotonin, which serve as cofactors for tyrosine hydroxylase (TH) and tryptophan hydroxylase. GTP cyclohydrolase (GCH) is the first and rate-limiting enzyme for BH4 biosynthesis.

What is a BH4 loading test?

The most used method is the BH4 loading test. Historically this test was performed at neonatal age, immediately after neonatal screening and aimed to distinguish hyperphenylalaninemic patients with PAH deficiency and patients with a BH4 deficiency [10, 11].

What does PKU smell like?

Symptoms of PKU Untreated children with phenylketonuria often give off a mousy or musty body odor in their urine and sweat. This odor is the result of phenylacetic acid, which is a by-product of phenylalanine.

What is BH4?

Tetrahydrobiopterin (BH4), also known as sapropterin (INN), plays a key role in a number of physiological processes. It is involved in the formation of neurotransmitters, heart and endothelial dysfunction, the immune response, and pain sensitivity [ 1 ]. BH4 is essential for the action of several enzymes, including ones responsible ...

What is BH4 supplement?

In mice, BH4 supplementation can help bring serotonin levels back to normal and steady digestive function [ 29 ].

What does BH4 do to the body?

The depletion of BH4 reduces the synthesis of neurotransmitters like serotonin, melatonin, dopamine, norepinephrine, and epinephrine. These compounds are essential for controlling daily body functions including mood, sleep cycle, memory, and appetite [ 25 ]. Loss of BH4 might explain some of the symptoms of depression.

What is BH4 bioavailability?

Reduction in vascular BH 4 bioavailability is a central mechanism for the development of impaired NO-mediated microvascular ...

Is BH4 dose dependent?

The effects of oral administration of BH4 are dose-dependent and further studies are required to investigate its long-term effects [ 9 ]. Additionally, the combination of BH4 with other antioxidant compounds is not yet tested [ 11 ].

Is BH4 a biological function?

BH4 is involved in other biological functions; however, no clinical evidence supports the use of supplemental BH4 for any of the conditions listed in this section. Below is a summary of the existing animal and cell-based research, which should guide further investigational efforts.

Is BH4 in cerebrospinal fluid lower in autism?

Levels of BH4 in cerebrospinal fluid are 42% lower in children with Autism Spectrum Disorder (ASD) [ 16 ]. It is possible that metabolic pathways that need BH4 are dysfunctional in children with ASD.

Why is BH4 important?

What Is BH4 and Why Is it Important in Phenylketonuria? In Health Insights. BH4 (tetrahydrobiopterin) plays an important role in the activity of the enzyme that is dysfunctional, or not working properly , in people with phenylketonuria (PKU). Here is more information about BH4 and how it may be able to help some PKU patients.

What is BH4 in PKU?

What is BH4 and how is it related to PKU? BH4 is a molecule that the body produces to act as a cofactor. A cofactor enables or enhances the activity of enzymes. One of the enzymes that works with BH4 is phenylalanine hydroxylase (PAH). This is the enzyme that is not present or does not work correctly in PKU.

What is the function of the PAH enzyme?

The PAH enzyme is normally responsible for converting the amino acid phenylalanine (Phe) into another amino acid, tyrosine. Amino acids are the building blocks of proteins. Without enough functioning PAH enzyme, Phe builds up in the body and leads to the symptoms of PKU.

How many pieces of a PAH enzyme are there?

The PAH enzyme consists of four identical pieces. If there is a fault in any of the pieces, they cannot fit together and work properly. BH4 may help these pieces fold correctly so that they can come together and form a more functional PAH enzyme. Since BH4 can regulate the activity of the PAH enzyme, researchers thought ...

Does BH4 interact with PAH?

Researchers don’t know the exact way in which BH4 interacts with the PAH enzyme. However, they think that it helps to stabilize the enzyme and keep it active. In normal conditions, BH4 concentration regulates the activity of PAH, with higher concentrations leading to more activity and more Phe conversion. A condition called tetrahydrobiopterin ...

The Basics

BH4 is an enzyme that is used to make serotonin, dopamine, thyroid hormones, melanin and to detox ammonia. It is recharged by folate and/or niacin and/or vitamin C. With certain combinations of the MTHFR gene, some people have a limited supply of BH4.

Reasons to Suspect

If you have low dopamine AND low serotonin AND low thyroid function (even subclinical). If you have high blood ammonia. If you have MTHFR polymorphisms.

Sources

The main way to increase BH4 is by recycling it with folate, niacin and/or vitamin C .

What is BH4 used for?

BH4, or tetrahydrobiopterin, is used for many imperative and fundamental processes in the body.

What is BH4?

BH4 is necessary in the production of neurotransmitters like serotonin, melatonin, dopamine, norepinephrine, and epinephrine, which help with body functions such as mood, sleep cycle, memory and appetite . If BH4 is low then it is possible for neurotransmitters to be low.

What is BH4 enzyme?

BH4 is a naturally occurring essential cofactor of the three aromatic amino acid hydroxlase enzymes used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin, melatonin, dopamine, norepinephrine, epinephrine, and is a cofactor for the production of nitric oxide by nitric oxide synthases. R

How long does it take for BH4 to decrease?

R. Ischemia (blood flow restriction, such as a stroke or hypoxia) reduces BH4 in the heart by 85-87% after 30-60 minutes of ischemia. R. Bh4 can help vascular flow, since depletion of BH4 can contribute to post-ischemic eNOS dysfunction.

What is the role of ammonia in cirrhosis?

By creating portosystemic shunts, cirrhosis allows absorbed ammonia to escape hepatic metabolism, increasing blood ammonia, which contributes to the pathogenesis of hepatic encephalopathy and can be neurotoxic. R.

Does BH4 cause gastroparesis?

This can be caused by a dysfunciton in endothelial nitric oxide synthase or eNOS. BH4 has a glucose-lowering effect. R BH4 can help gastroparesis. R. BH4 leads to less glucose intorance and insulin resistance and has the potential to alleviate symptoms of diabetes. R R.

Can BH4 cause phenylketonuria?

BH4 deficiency can cause phenylketonuria, which is the buildup of excess phenylalanine in the body. R. This can cause severe brain damage, mental retardation, seizures, and other abnormalities. R. BH4 may help lower phenylalanine levels in some people with phenylketonuria. R.

What is BH4 deficiency?

Tetrahydrobiopterin (BH4) deficiency is a neurological condition characterized by abnormalities in the production and regeneration of tetrahydrobiopterin. Tetrahydrobiopterin, a naturally occurring chemical substance, which helps to enhance the functions of certain enzymes. Deficiency of BH4 leads to abnormally high levels of the amino acid, ...

How does BH4 affect tetrahydrobiopterin?

Mutation in any of these genes affects the production and regeneration of tetrahydrobiopterin. Tetrahydrobiopterin has the following function in our bodies: BH4 deficiency is acquired in an autosomal recessive manner, which means the disease will manifest when both the copies of the gene obtained from parents have mutations.

How many chances are there of having a child with BH4?

When two carriers of an autosomal recessive condition have children, there is a 25% chance to have a child with BH4 deficiency.

What are the functions of tetrahydrobiopterin?

Tetrahydrobiopterin has the following function in our bodies: 1 Breaking down or processing certain amino acids 2 Proper development of amine neurotransmitters, such as catecholamines and serotonin

What is the cofactor of BH4?

Tetrahydrobiopterin. Tetrahydrobiopterin ( BH4, THB ), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), ...

What is the function of tetrahydrobiopterin?

The major one is to convert amino acids such as phenylalanine, tyrosine, and tryptophan to precursors of dopamine and serotonin, major monoamine neurotrans mitters. It works as a cofactor, being required for an enzyme's activity as a catalyst, mainly hydroxylases.

What is the function of phenylalanine hydroxylase?

Phenylalanine hydroxylase (PAH) catalyses the conversion of L-phenylalanine (PHE) to L-tyrosine (TYR). Therefore, a deficiency in tetrahydrobiopterin can cause a toxic buildup of L-phenylalanine, which manifests as the severe neurological issues seen in phenylketonuria .

When was tetrahydrobiopterin approved?

It was approved for use in the United States as a tablet in December 2007 and as a powder in December 2013.

Is tetrahydrobiopterin a therapeutic target?

Since nitric oxide production is important in regulation of blood pressure and blood flow, thereby playing a significant role in cardiovascular diseases, tetrahydrobiopterin is a potential therapeutic target.

Is BH4 safe for autism?

In 1997, a small pilot study was published on the efficacy of tetrahydrobiopterin (BH4) on relieving the symptoms of autism, which concluded that it "might be useful for a subgroup of children with autism" and that double-blind trials are needed, as are trials which measure outcomes over a longer period of time. In 2010, Frye et al. published a paper which concluded that it was safe, and also noted that "several clinical trials have suggested that treatment with BH4 improves ASD symptomatology in some individuals."

What Is Tetrahydrobiopterin (Bh4)?

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Potential Health Benefits of BH4

Side Effects, Interactions, & Dosage