Organic Acid Test, Part 7: Amino Acid Metabolites

Please note: The content of this post is my own unless the technical terms are too hard to explain, then the content is copy and paste. I am not a medical professional and thus the post is my point of view. But the content is science-based and credible. Just because it is a new science, does not necessarily make it quackery.

Test results, part 4

Amino Acids

What is an amino acid, you ask? Amino acids are used in every cell of your body to build the proteins you need to survive. The best sources of essential amino acids are animal proteins like meat, eggs and poultry.

In the Great Plains Organic Acid test, there are about 15 markers that are tested for regarding amino acids. In the SA test, it is broken up into two categories.

Urine organic acids: Branched-chain amino acid intermediates

2-Ethylhydracrylic-/2-Ethyl-3-OH-propionic acid 0.35 < 2 mmol/mol creat
2-Hydroxyisocaproic acid 0.22 < 2 mmol/mol creat
2-Hydroxyisovaleric acid BDL < 2 mmol/mol creat
2-Oxoisovaleric acid / 3-Methyl-2-oxobutyric acid 0.22 < 1.1 mmol/mol creat
3-Hydroxy-2-methylbutyric acid 0.85 < 11 mmol/mol creat
3-Hydroxyisobutyric acid 5.48 L 20 – 118 mmol/mol creat
3-Hydroxyisovaleric acid 5.51 5.1 – 23.1 mmol/mol creat
3-Methyl-2-oxovaleric-/2-Keto-3-methylvaleric acid BDL < 4.8 mmol/mol creat
3-Methylglutaconic acid 2.89 < 20 mmol/mol creat
3-Methylglutaric acid 0.99 < 3.9 mmol/mol creat
4-Methyl-2-oxovaleric acid/2-Ketoisocaproic acid 0.16 < 0.86 mmol/mol creat
Malonic acid BDL < 5 mmol/mol creat

Urine organic acids: Other Amino acid intermediates

3-Hydroxyglutaric acid (Lysine Metabolism) 2.05 < 8 mmol/mol creat
Glutaconic acid (Lysine Metabolism) 0.58 < 3.1 mmol/mol creat
N-Acetylaspartic acid  (Aspartic Metabolism) 0.54 < 13 mmol/mol creat

This one is a bit more difficult to break down.

According to the Great Plains Lab break down:

2-Hydroxyisovaleric Acid

2-Oxoisovaleric Acid

3-Methyl-2-oxovaleric Acid

2-Hydroxyisocaproic Acid

2-Oxoisocaproic Acid

A moderate increase of branched-chain amino acid metabolites in urine may result from lactic acidosis, episodic ketosis, or deficiencies of the vitamins thiamine or lipoic acid.  Elevated 2hydroxyisocaproic acid in urine has also been linked to short bowel syndrome.  A significant increase of branched-chain amino acid metabolites is associated with the genetic disorders maple syrup urine disease (MSUD) or pyruvate dehydrogenase deficiency.  Patients with slight to moderate elevations may use dietary supplements containing thiamine to improve clinical symptoms.

2-Oxo-4-methiolbutyric Acid      Testing of plasma amino acids.        Elevated in an inborn error of methionine metabolism.  Confirmation of the genetic disorder requires

Malonic Acid Associated with the genetic disorders malonyl-CoA decarboxylase deficiency or malonic aciduria with normal malonyl-CoA decarboxylase activity.  Slightly elevated values in urine are unlikely to be clinically significant.

3-Methylglutaric Acid and 3-Methylglutaconic Significant increase are due to a reduced ability to metabolize the amino acid leucine.  This abnormality is found in the genetic disease methylglutaconic aciduria and in mitochondrial disorders.  3-Methylglutaconic acid may also be elevated.  Supplementation with coenzyme Q10, NAD+, L-carnitine and acetyl-L-carnitine, riboflavin, nicotinamide, biotin, and vitamin E may be useful.

Amongst the list were also markers found under Neurotransmitters which include Phenylalanine and Tyrosine Metabolites. But more about those in the next post.

So any reading in this section was the 3-Hydroxyisobutyric acid, which was low.

3-Hydroxyisobutyric acid (or 3-hydroxy-2-methylpropanoic acid) is an intermediate in the metabolism of Valine.

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Just for fun: the chemical structure of 3-Hyrdoxyisobutyric acid

An interesting fact about Valine, it was first isolated from casein in 1901 by Hermann Emil Fisher. The name valine comes from valeric acid, which in turn is named after the plant valerian due to the presence of the acid in the roots of the plant. Casein which is present in milk.

This result should be right because Alex does not drink milk or eat any dairy products, not because he is allergic to it or on a casein-free diet, but because he does not like it.

Dr Lindenberg comments: “Branched-chain amino acids OK with relatively low tyrosine metabolism.”

Interesting read: An Amino-Acid Deficiency That Causes Neurological Problems

There is a condition called 3-hydroxyisobutyric aciduria. In many affected people, the exact underlying cause of 3-hydroxyisobutyric aciduria is poorly understood. Scientists believe that some cases are caused by changes (mutations) in the ALDH6A1 gene. This gene encodes an enzyme called methylmalonate semialdehyde dehydrogenase, which helps the body break down certain amino acids (the building blocks of protein) found in food. If this gene isn’t working properly, the body is unable to break down the amino acids valine and thymine which leads to a build-up of toxic substances in the body and the many signs and symptoms of 3-hydroxyisobutyric aciduria. There is no cure for 3-hydroxyisobutyric aciduria. Because it is so rare, there is limited evidence to support the effectiveness of treatment. However, affected people have been treated with a protein-restricted diet and carnitine supplementation with varying degrees of success. (https://rarediseases.info.nih.gov/diseases/5662/3-hydroxyisobutyric-aciduria

 

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