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pawofdoom671 karma

By lying about it

pawofdoom296 karma

tldr: You are producing epinepherine - you'd be extremely dead if you weren't. The creation pathway to neurotransmitters is quite separate from your listed hormones.

Adrenaline (medically known as epinephrine) is produced from noradrenaline (norepinephrine) which is produced from dopamine... from l-dopa... from tyrosine... from phenylalanine (an amino acid) and the catalyst (sorta) tetrahydrobiopterin ("BH4").

Each stage and many additional ones involved in the initial production and recycling of BH4 - after its been oxidized to BH2 when creating tyrosine - requires a specific enzyme to do the job. Any genetic problems in any of these enzymes usually causes severe and immediately apparent conditions in babies, the most common being "PKU".

Patients with PKU usually have a faulty phenylalanine hydroxylase enzyme which converts BH4 + phenylalanine into tyrosine. Unmanaged patients have extremely high levels of phenylalanine in the blood (as they can't get rid of it) and tyrosine deficiencies. Untreated, mental deficiency is inevitable. Treatment for ~20% of patients involves BH4 supplementation via "Kuvan" (an artificial form of BH4 as natural BH4 can't cross the blood brain barrier) and a very low protien diet. The rest of PKU patients will live on a near 2g protien allowance diet, with the rest provided by engineered protien which doesn't include phenylalanine.

Other enzyme disorders are much rarer and most are ~1/1,000,000 type rarity. Some cause PKU, some cause symptoms via dopamine / serotonin deficiency and others are just plain complicated. Most cause severe disability when not treated straight away, others do okay. Even rarer variants of the variants involve complicated adolescence or adult onset which involve lots of borderline "just enough" levels of these neurotransmitters which lapse into insufficiency.

I write this from memory while needing to go so I hope I got the spellings right.


Edit: Not a doctor, not your doctor, not anyone's doctor.

Edit2: Yay gold, now I am a third of an Adam Savage /u/mistersavage! Maybe I can save up enough coins to one day also be able to touch Jamie's mustache without being turned to ballistics gel.

pawofdoom61 karma

Just a coincidence that I've recently spent weeks researching the pathway and relavent conditions from end to end. I feel like I could recite parts of "The Physician's guide to ... Metabolic Diseases", which is an excellent text as both a comprehensive resource and quick reference guide.

This is my favorite image for describing the pathway the simplest and I like this one for adding in the positions of the relevant diseases.

pawofdoom35 karma

I'm sure you can get it. On the second image:

  • The black texts circled in green are the four neurotransmitters we want to make.
  • The dark blue texts are the enzymes that break them down and what byproducts we get from them.
  • The middle two circles (overlapping) are the BH4 regeneration cycle in red and the two main reactions where BH4 is used in green.
  • BH4 is used to create tyrosine from phenylalanine (not actually shown), to turn tyrosine into l-dopa, and separately to turn typtophan into 5-HTP.
  • When that happens, the BH4 is oxidised to BH2, so we need to do some work on it to get it back to the BH4 form we want. This is the red circle. In the process, we create some other byproducts.
  • The top blue process is what we have to do to turn GTP - one of the core molecules that powers all cells - into BH4. It takes many different enzymes many steps to get to there.
  • Underlined abbreviations are the enzyme that performs the reaction.
  • Circled numbers are the conditions arrising when their corresponding enzyme is mis-coded or missing.

pawofdoom19 karma

So to speak as the devil's advocate for those unwilling to write a reply; that's a very unique situation which only a handful of people can match. While you essentially retained a full time job in terms of time, energy and emotional commitments, you didn't have to live. Gone are the days that one can rent a garage and live on $100 a month; we now need full time jobs to feed ourselves. My point being almost no one can grind through unsuccessfulness because it doesn't prevent you starving.