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Palmitoleic acid ((Z)-Hexadec-9-enoic acid) is found to be associated with isovaleric acidemia, which is an inborn error of metabolism.

Malonic acid (propanedioic acid) is the archetypal example of a competitive inhibitor: it acts against succinate dehydrogenase (complex II) in the respiratory electron transport chain. Malonic acid is found to be associated with malonyl-CoA decarboxylase deficiency, which is an inborn error of metabolism.

It is an isomer of cresotinic acid (2-hydroxy-3-methylbenzoic acid) and oxymethylbenzoic acid (2-methoxybenzoic acid). Derivatives of mandelic acid are formed as a result of metabolism of adrenaline and noradrenaline by monoamine oxidase and catechol-o-methyl transferase. It is also present in certain skin care products, is an intermediate molecule in the production of other biochemicals, may be used as an analytical reagent and is a precursor in the manufacture of dyes. Mandelic acid (acidomandelico) is found to be associated with phenylketonuria, which is an inborn error of metabolism.

Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239 ). Methylglutaric acid is also found to be associated with 3-hyd

Increased levels of erucic acid (22:1n9) have been found in the red blood cell membranes of autistic subjects with developmental regression (PMID: 16581239 ). Erucic acid (13(Z)-Docosenoic Acid) is broken down long-chain acyl-coenzyme A (CoA) dehydrogenase, which is produced in the liver. This enzyme breaks this long chain fatty acid into shorter-chain fatty acids. human infants have relatively low amounts of this enzyme and because of this, babies should not be given foods high in erucic acid. Erucic acid is found to be associated with isovaleric acidemia, which is an inborn error of metabolism.

3-Hydroxyphenylacetic acid is a rutin metabolite and an antioxidant. It has a protective biological activity in human. It is a substrate of enzyme 4-hydroxyphenylacetate 3-monooxygenase [EC 1.14.13.3] in the pathway tyrosine metabolism. 3-Hydroxyphenylace

Phenylpyruvic acid (3-Phenylpyruvic acid) is a keto-acid that is an intermediate or catabolic byproduct of phenylalanine metabolism. It has a slight honey-like odor. Levels of phenylpyruvate are normally very low in blood or urine. High levels of phenylpyruvic acid can be found in the urine of individuals with phenylketonuria (PKU), an inborn error of metabolism.

2,2-Dihydroxyacetic acid (Formylformic acid) is an intermediate in the glyoxalate cycle, which enables certain organisms to convert fatty acids to carbohydrates.2,2-Dihydroxyacetic acid has been found to be associated with primary hyperoxaluria, an inborn error of metabolism. As an aldehyde, glyoxalate is also highly active and can modify proteins to form advanced glycosylation products (AGEs).

3-Amino-2-methylpropanoic acid (α-Methyl-β-alanine) is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6), the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities.

Dihydroxyacetone phosphate is an important intermediate in lipid biosynthesis and glycolysis, involved in various metabolic pathways, including the Calvin cycle and glycolysis in plants. It is also associated with the lack of transaldolase, a congenital metabolic error.

4-Hydroxyphenylpyruvic acid is an enzyme inhibitor.4-Hydroxyphenylpyruvic acid (4-HPPA) is a keto acid that is involved in the tyrosine catabolism pathway. It is a product of the enzyme (R)-4-hydroxyphenyllactate dehydrogenase (EC 1.1.1.222) and is formed during tyrosine metabolism. The conversion from tyrosine to 4-HPPA is catalyzed by tyrosine aminotransferase. Additionally, 4-HPPA can be converted to homogentisic acid which is one of the precursors to ochronotic pigment. The enzyme 4-hydroxyphenylpyruvic acid dioxygenase (HPD) catalyzes the reaction that converts 4-hydroxyphenylpyruvic acid to homogentisic acid. A deficiency in the catalytic activity of HPD is known to lead to tyrosinemia type III, an autosomal recessive disorder characterized by elevated levels of blood tyrosine and massive excretion of tyrosine derivatives into urine. It has been shown that hawkinsinuria, an autosomal dominant disorder characterized by the excretion of 'hawkinsin,' may also be a result of HPD deficiency . Moreover, 4-hydroxyphenylpyruvic acid is also found to be associated in phenylketonuria, which is also an inborn error of metabolism. There are two isomers of HPPA, specifically 4HPPA and 3HPPA, of which 4HPPA is the most common.