Ask Beth: “Do you think there are instances where people do not process chemicals properly and therefore need support from supplements to aid in the processing of them?”

Sometimes I get asked good questions from friends with inquiring minds. I thought I would share them in case anyone is asking these same questions.

To answer this question, I will break it up into 2 parts:

1. “Are there instances where people do not process chemicals properly?”

Yes, there certainly are, and unfortunately, this results in a lot of disease conditions. For example, people may be born with a genetic mutation where a gene that makes one of the hundreds of enzymes in mitochondria that does not function properly. The enzyme may be responsible for a chemical reaction to make something or break something down. They are what “process chemicals”.  If you lack the enzyme that makes something, then you will lack that product. If you lack an enzyme key in breaking things down, the byproducts of that specific metabolic pathway will build up and can be toxic. There are an array of mitochondrial diseases. These are disease, and if you are missing an enzyme because of a genetic mutation, you would have serious symptoms, and in some cases if not treated, will cause death. Many of these mitochondrial diseases can cause bleeding in the brain and present as child abuse (without a positive skeletal survey revealing broken long bones or ribs common in infants who have brain bleeding due to child abuse), which is another interest of mine.

Here’s one example:

  • Propionyl coenzyme A carboxylase deficiency

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Figure 1. A very beautiful 3D schematic of Propionyl coenzyme A carboxylase. The alpha and beta subunits move and interact together to “do chemistry”.

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Figure 2. The work propionyl coenzyme A does in your mitochondria all day long without you taking a vitamin supplement.

  • Inheritance Autosomal recessive.This is a ‘mitochondrial’ disease since the deficient enzyme – propionyl coenzyme A carboxylase – is located within the mitochondria. Encoding genes have been found on both chromosome 3 (beta subunits) and 13 (alpha subunits).
  • Metabolic features: The neonatal form presents with increased ammonia levels, erroneously suggesting a urea cycle defect. Ketosis, acidosis (metabolic and lactic) and hypoglycemia are found in conjunction with increased glycine levels (see above in methylmalonic acidemia). For the aforementioned reasons the disease is also called ‘ketotic hyperglycinemia’. The co-factor of the enzyme is biotin; hence other enzyme deficiencies (multiple carboxylase deficiency, see later in 3-methylcrotonyl coenzyme A carboxylase deficiency) related to impairment of the biotin cycle (holocarboxylase synthetase, biotinidase) may cause diagnostic problems.
  • Clinical features: The disease has a severe neonatal (80–90%) and a milder infantile (10–20%) onset variant. Skin rashes, hypotonia, lethargy, dehydration, seizures and irregular breathing are seen in the neonate before severe acidosis leads to coma and death.
  • Clinical course: Prognosis in the late-onset form is much better. If the ketoacidotic metabolic crises which are often triggered by infection, fasting, constipation or high protein intake are successfully prevented, affected patients may reach adult age.
  • Treatment: Low protein diet with l-carnitine supplementation. (copied from http://www.mrineonatalbrain.com/ch04-17.php)

2. “… and therefore need support from supplements to aid in the processing of them?”

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Figure 3. A bobcat.

Enzymes are a type of protein and function as the work horses in our body. Let’s use an analogy that bobcat is one type of enzyme/protein- it has a 3D shape, it moves in space, does work, “processes chemicals”, vitamins are like the oil that keeps the bobcat running, and ATP is the gasoline. Vitamins lower the energy required for the enzyme to work – less gasoline/ATP. How do we get our enzymes? Sometimes  “food mysticism” literature (internet), implies that we are passive to our food and that we can digest key enzymes in raw milk or raw food. However, our GI tract does not absorb proteins/enzymes. The pH of the stomach is around 4 and then peptidases in the small intestine rip enzymes/proteins apart. Our intestine actually absorbs amino acids- sometimes as long as 3 amino acids in a row, but not proteins. Going back to our bobcat analogy, if we eat a bobcat, which is a protein in a steak, it will get homogenized in our stomach and then in our small intestine peptidases chop up the bobcat into parts- the fender, the tire, the wheel, the smokestack and then specific transporters for each part move it across the gut epithelia into our body. The inside of our small intestine is actually “outside” of our body, which is good as it has billions of bacteria (another interesting topic). Your gut epithelia is like the Berlin Wall- only very specific things can pass through. Also, vitamins pass through via their specific transporters and each type of nutrient gets across very specific transporters. What does your body do with these bobcat parts? The bobcat parts get absorbed into your blood and diffuse across capillaries when in your tissue, let’s say your bicep has a low concentration of arginine (an amino acid) as it’s constantly turning over muscle. Arginine, a bobcat part- let’s say a tire, would be transported into the muscle cell and if your muscle cell is deciding to make bobcats that day, it’s DNA, which is transcribed and then translated would instruct the cell to add arginine (a tire) to a bobcat it’s making or maybe another protein, let’s say it’s making myosin that day… a corvette. It would pop that tire on from the bobcat it ripped apart in your small intestine and then put it on a corvette in the cell in your bicep.

Our bodies are incomprehensibly complex and are robust factories that make very specific products on it’s own very specific agenda due to orders and communications from within the cell, the cell’s neighbors, hormones from the gut, hormones from the brain etc.. It is not passive. We eat food, our body rips it apart, and then decides what it wants to make with the parts. Of course, if you have a genetic mutation in an enzyme, including enzymes that process vitamins, you are going to have problems.

If you were unable to make a bobcat… or a corvette.. or any other key protein, you would have a disease. There is a very large area of scientific inquiry to try to solve the problem of how to correct mutated genes to make proteins we need. Sometimes it’s as simple as giving more of the protein that is missing (or being destroyed by your body)- like giving insulin in diabetes. Mitochondrial disease or any other metabolic disease, there is a huge effort to learn how to genetically engineer cells to give them the correct DNA to make the right protein (or bobcat) themselves. I have a friend who is a PhD student at MIT working in one of these labs doing very crazy theoretical things with DNA so that these disease where a protein (the parts of our cells that process chemicals) that is defective is corrected at the level of the DNA.

A big part of “food mysticism” is that vitamins are given more power than they have. You could give as much vitamins as you want to a person with a mitochondrial disease, but a.) if you one of your proteins isn’t working you have a disease, b.) if your DNA makes bobcats without pistons- no amount of oil is going to help. People with mitochondrial disease can manage their diet to decrease the activation of pathway involving the defective enzyme, but you can pour oil on your broken bobcat all day and it will not work. “Food mysticism” usually mixes in things that are correct: yes, it is possible that some people may not process chemicals properly, and yes, we do need vitamins +  incorrect information to get you to buy something (or click on a website which generates add revenue), but incorrect processing would lead to disease which would need a lot more treatment then the vitamins they are trying to sell you.

In conclusion, if you have a protein that’s not doing it’s job correctly, you have a disease that would need to be treated with medicine or altering your diet to avoid that metabolic pathway so you don’t die. Vitamins don’t help these conditions.

Note: Vitamin D is really the only vitamin that is found to be deficient in people not living in third world country based on scientific evidence— especially people living in regions with long winters. Though vitamin deficiency is obviously a problem, it does not mean that adding more vitamins is helpful if you are not deficient and vitamin supplements are actually associated with increased incidence of cancer and other problems.

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