What is 2-Hydroxybutyric acid? 2-Hydroxybutyric acid (aka α-Hydroxybutyrate) is a by-product of glutathione production. Levels of 2-Hydroxybutyric acid in the urine may reflect levels of glutathione production. What is Glutathione? Glu

2-Hydroxyhippuric acid is a conjugate of hydroxybenzoic acid (salicylic acid) and glycine.

2-Hydroxyisocaproic acid (aka Leucic acid / α-hydroxyisocaproic acid / HICA) is a metabolite of the branched-chain amino acid leucine.

2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite. 2-hydroxy-3-methylbutyric acid is a valine derivative that is valine in which the amino group has been replaced by a ‘hydroxy’ gro

The metabolite of phenylalanine via phenyl pyruvate.

2-Oxo-4-methylthiobutanoic acid, also known as 4-(methylsulfanyl)-2-oxobutanoate or 2-keto-4-methylthiobutyrate, belongs to the class of organic compounds known as thia fatty acids. Thia substituted fatty acids are saturated 

2-Oxoglutaric acid is an organic acid that is important for the proper metabolism of all essential amino acids. It is formed in the Krebs cycle, the energy-producing process that occurs in most body cells.

2-Oxoisocaproic acid (also known as Ketoleucine) is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 2-Oxoisocaproic acid is both a neurotoxin and a metabotoxin. A moderate increase of branche

- A precursor in the production of cholesterol in both humans and yeast. - Elevated levels may also indicate decreased CoQ10 synthesis. - Moderate increases are probably due to yeast overgrowth of the GI tract and might also implicate yeast ove

3-Hydroxybutyric acid is a typical partial-degradation product of branched-chain amino acids (primarily valine) released from muscle for hepatic and renal gluconeogenesis. This acid is metabolized by 3-hydroxybutyrate dehydrogenase. The enzyme f

3-Hydroxyglutaric is a marker for glutaryl CoA dehydrogenase deficiency.

3-Methyl-2-oxovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids.  Moderate increase may result from lactic acidosis, episodic ketosis, or thiamine/lipoic acid deficiency. Significan

The marker, 3-methylglutaconic acid in high values indicates a reduced ability to metabolize the amino acid, leucine. This abnormality is found in the genetic disease, methylglutaconic aciduria and in mitochondrial disorders.

3-methylglutaric acid is an organic acid classically associated with two distinct leucine pathway enzyme deficiencies.

A moderate urinary increase in 4-hydroxybutyric acid may be due to intake of dietary supplements containing 4-hydroxybutyric acid, also known as gamma-hydroxybutyric acid. Very high levels may indicate the genetic disorder 3-methylglutaconic aciduria

5-Hydroxymethyl-furoic Acid is a naturally occurring human urinary metabolite, a nematicide, a bacterial xenobiotic metabolite and a fungal metabolite. It is a metabolite produced by Aspergillus and possibly other fungal species in the GI tract. Cons

Acetoacetic acid (=acetoacetate) is a ketone body and a weak Beta-keto acid produced from acetyl-CoA in the mitochondrial matrix of hepatocytes.

Elevated in mitochrondrial disorders. Aconitase metabolizes citric and aconitic acids, and is dependent on glutathione.

Adipic Acid, together with Suberate and Ethylmalonate are all functional markers for deficiency of carnitine.

Breakdown product of hyaluronic acid; also found in certain foods.

Ascorbic Acid is a natural water-soluble vitamin (Vitamin C). Ascorbic acid is a potent reducing and antioxidant agent that functions in fighting bacterial infections, in detoxifying reactions, and in the formation of collagen in fibro

Citric acid, cis-aconitic acid, and isocitric acid are the first three metabolites in the Krebs Citric Acid energy production cycle, which operates in the mitochondria of your cells. 

Ethylmalonate, together with Adipate and Suberate, gives information about your ability to process fatty acids.

Fumarate (together with Succinate and Malate) is used in the body’s metabolic pathway that generates cellular energy – the Citric Acid Cycle.

Furancarbonylglycine is a metabolite produced by Aspergillus and possibly other fungal species in the GI tract.

γ-amino butyric acid (GABA) is the major inhibitory neurotransmitter of the brain. Its major precursor is L-glutamate, which is converted to GABA via the enzyme glutamate decarboxylase (GAD). GABA has also been detected in other peripheral t

Glutaric Acid is formed from the essential amino acids lysine and tryptophan through the intermediaries of alpha ketoadipic acid and glutaryl-CoA. Glutaryl-CoA is further metabolized to glutaconyl- and crotonyl-CoA by an enzyme called glutaryl-CoA de

Glyceric acid is an organic acid that stems from the catabolism of the amino acid serine. Severe elevations in glyceric acid are an indication of a rare inborn error of metabolism known as glyceric aciduria. One form of glyceric aciduria is the resul

Benzoic acid and hippuric acid are formed from the bacterial metabolism of polyphenols. Urinary benzoic acid may also come from ingestion of food preservatives such as sodium benzoate. Hippuric acid is made when sodium benzoate is conjugated with gly

Homovanillic acid (HVA) is a major catecholamine metabolite that is produced by a consecutive action of monoamine oxidase and catechol-Omethyltransferase on dopamine. HVA is associated with dopamine levels in the brain. Dopamine (3,4-dihydroxypheneth

HPHPA is a clostridia metabolite and dysbiosis marker and stands for 3-(3-hydroxyphenyl)-3-hydroxypropionic acid. HPHPA is an indicator for dysbiosis due to overgrowth of Clostridia bacteria (e.g., C. sporogenes, C. caloritolerans,

HVA and DOPAC are the major metabolites of dopamine. HVA stands for Homovanillic acid and DOPAC stands for Dihydroxyphenylacetic.

Lactic acid is a microbial metabolite, urinary lactic acid is produced by Escherichia coli, Pseudomonas aeruginosa, Klebsiella pneumonia, Enterobacter, Acinetobacter, Proteus mirabilis, Citrobacter frundii, Enterococcus faecalis, Streptococcus group

Fumaric acid uses the fumarase enzyme to become malic acid. Malate dehydrogenase catalyzes the conversion of malic acid into oxaloacetate. Two forms of this enzyme exist in eukaryotes. One operates within the mitochondria to contribute to the Citric

Methylcitric is an organic acids that reflects decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase.

Methylmalonic acid (MMA) is a substance produced in very small amounts and is necessary for human metabolism and energy production.

Methylsuccinic acid is a normal metabolite found in human fluids and is an intermediate metabolite in the breakdown of fatty acids.

Elevated N-acetylaspartic acid is due to the genetic disorder Carnavan’s disease, a potentially fatal disease causing spongy degeneration of the brain.  What is Carnavan’s disease? Canavan disease is a rare inherited disorder t

N-Acetylcysteine (NAC) is a powerful antioxidant that increases the glutathione reserves in the body.  Together with glutathione, N-Acetylcysteine (NAC) directly binds to toxic metabolites. Although N-Acetylcysteine (NAC) may be benefici

Pantothenic acid is an essential B vitamin (=Vitamin B5) that is converted to coenzyme A (unrelated to vitamin A). Coenzyme A is needed for the synthesis of fatty acids, cholesterol, and acetyl choline and is also needed for the Krebs cycle and fatty

A major metabolite of vitamin B6. High pyridoxic acid indicates high recent intake of vitamin B6. Because some individuals may require very high doses of vitamin B6, high values do not necessarily indicate the need to reduce vitamin B6 intake. Low va

Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. Pyruvic acid is found to be associated with Fumarase deficiency, which is an inborn error of metabolism. It is also a metabolite of Corynebacterium. Elev

A high ratio of quinolinic acid to the tryptophan metabolite, 5-hydroxyindoleacetic acid, indicates excessive inflammation due to recurrent infections, excessive tryptophan intake, immune overstimulation, excessive adrenal production of cortisol, or

Increased urinary products of the omega fatty acid metabolism pathway may be due to carnitine deficiency, fasting, or increased intake of triglycerides from coconut oil, or some infant formulas.

- Suberic acid is present in the urine of people with fatty acid oxidation disorders. - A metabolic breakdown product derived from oleic acid. - Elevated levels of this unsaturated dicarboxylic acid are found in individuals with medium-chain ac

Succinyl CoA becomes succinic acid using succinyl CoA synthetase. This reaction produces NADH which directly provides electrons for the electron transport chain or respiratory chain. Succinic acid requires the enzyme succinate dehydrogenase to become

A pyrimidine (DNA building block) that is elevated in the genetic disease dihydropyrimidine dehydrogenase deficiency. In this genetic disease, the pyrimidine uracil is also elevated. - Thymine is one of the five bases used to build nucleic acids.

The pyrimidine metabolites are markers of folate metabolism. The two markers are uracil and thymine. Folate acts as a methyl donor in converting uracil to thymine. Elevated values of uracil suggest folic acid deficiency. Folate is needed to conver