Is there BH4 supplement/medicine?

[GlassCannonLife]

As an update, I have been on a 20mg dose. I was able to provide by insurance with amino acid results that showed high phenylalanine which convinced them I should get it. BioMarin also had a group who can help you get it approved.

Do you have to take it ongoingly to maintain the benefits?

 

[Alvin2]

Any updates on this?
I'm also in Canada. Not sure i can get the doc to prescribe it and its not covered by my drug plan unless i have PKU (which afaik i do not).

 

[Consul]

https://www.omfcanada.ngo/genetic-and-metabolic-markers-of-bh4-deficiency-in-long-covid/

STUDY HYPOTHESIS AND DESCRIPTION
Tetrahydrobiopterin (BH4) is a vitamin-like cofactor that is made in the body by a series of enzymatic steps. However, there are common mutations in the biosynthesis pathway that are associated with cofactor deficiency. BH4 is needed for metabolism of a small number of amino acids that are precursors to neurotransmitters, and deficiency leads to cognitive impairment and a wide variety of other symptoms associated with neurotransmitter imbalance. BH4 is also needed for the enzyme nitric oxide synthase, which is a major regulator of blood flow dynamics and immune cell function.

We have determined that individuals with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome are more likely to have pathogenic variants in BH4 synthesis genes compared to the general population, and we have also identified a corroborating metabolic signature that predicts BH4 deficiency. Oxidative stress during infection can also lead to BH4 deficiency, so there are many potential routes to the same problem. We propose to test whether these genetic markers and other indicators of BH4 deficiency are also present in subjects with Long Covid. If we are successful, we will show that ME/CFS and Long Covid have a common underlying disease mechanism that includes deficiency of BH4.

Ron Davis study

 

[Alvin2]

https://www.omfcanada.ngo/genetic-and-metabolic-markers-of-bh4-deficiency-in-long-covid/



Ron Davis study

Is this new?
I would totally sign up for this if i knew how.

On my own front i tried to find out what pharmacy stocks BH4 it and the price (assuming i can get a prescription), its about $50/tablet and my local pharmacy can't get it.
My drug plan does not cover the cost except for PKU and even then its by application/approval only.

Though apparently there is a generic on the market since 2020ish in Canada, the pharmacy never looked for the generic so i have to call again and get them to look into it.

 

[Consul]

Is this new?
.

Not sure. But they apparently found more dysfunctional mutations in the relevant genes in pwme than controls, that sounds like news to me. Big news i would say.

 

[Alvin2]

Not sure. But they apparently found more dysfunctional mutations in the relevant genes in pwme than controls, that sounds like news to me. Big news i would say.

I hope its big news but tragically genetic deviations are a dime a dozen and most of them mean very little.
On the plus side if we can compensate by taking BH4 thats good news.
And hopefully they investigate it and either figure out it is a big deal or label it as noise.

 

[pattismith]

https://www.omfcanada.ngo/genetic-and-metabolic-markers-of-bh4-deficiency-in-long-covid/



Ron Davis study

I wish I could read the full paper!

 

[Consul]

New pilot study from Simmaron research finds high levels of BH4 instead of low levels.

https://www.mdpi.com/1422-0067/24/10/8713

 

[Violeta]

New pilot study from Simmaron research finds high levels of BH4 instead of low levels.

https://www.mdpi.com/1422-0067/24/10/8713

They should measure the neurotransmitters because maybe Bh4 isn't being used to form neurotransmitters and is just floating around in the blood stream.

"BH4 is an important cofactor of amino acid metabolism [8]. Amino acids, such as phenylalanine, tyrosine, and tryptophan, are converted into important cellular intermediates by the action of hydroxylase enzymes. BH4 serves as an essential cofactor of all these hydroxylase enzymes [9,10,11]. Accordingly, the bioavailability of BH4 is critical for the synthesis of neurotransmitters, such as dopamine and serotonin [10]. Apart from these hydroxylase enzymes, BH4 also regulates the function of nitric oxide synthase enzymes and controls the production of nitric oxide (NO) in endothelium."

Are they going to go anywhere with the oxidative stress mentioned in the article?

"Finally, a ROS production assay in cultured microglial cells followed by Pearson correlation statistics indicated that the elevated BH4 in serum samples of CFS + OI patients might be associated with the oxidative stress response."