3-Hydroxypropionic acid is a carboxylic acid. It is an intermediate in the breakdown of branched-chain amino acids and propionic acid from the gut. Typically it originates from propionyl-CoA and a defect in the enzyme propionyl carboxylase. This leads to a buildup in propionyl-CoA in the mitochondria. Such a buildup can lead to a disruption of the esterified CoA:free CoA ratio and ultimately to mitochondrial toxicity. Detoxification of these metabolic end products occurs via the transfer of the propionyl moiety to carnitine-forming propionyl-carnitine, which is then transferred across the inner mitochondrial membrane. 3-Hydroxypropionic acid is then released as the free acid. As an industrial chemical, it is used in the production of various chemicals such as acrylates in industry. When present in sufficiently high levels, 3-hydroxypropionic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of hydroxypropionic acid are associated with many inborn errors of metabolism including biotinidase deficiency, malonic aciduria, methylmalonate semialdehyde dehydrogenase deficiency, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, and propionic acidemia. Hydroxypropionic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis.
Sources:
Possible causes:
- Low B12 (methylmalonic acid will be high)
- Low biotin &/or Mg
- Inborn errors of metabolism
Additional investigations:
- Red blood cell B12
Treatment considerations:
- Supplement vitamin B12, biotin &/or Mg
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2-Hydroxyphenylacetic Acid, 3-Hydroxyisovaleric Acid, 3-Hydroxyphenylacetic Acid, 3-Hydroxypropionic Acid, 3-Methyl-4-OH-phenylglycol, 4-Hydroxyphenylacetic Acid, 5-OH-indoleacetic Acid, a-Hydroxyisobutyric Acid (from MTBE), a-Keto-b-Methylvaleric Acid, a-Ketoadipic Acid, a-Ketoglutaric Acid (AKG), a-Ketoisocaproic Acid, a-Ketoisovaleric Acid, a-Ketophenylacetic Acid (from Styrene), Adipic Acid, Arabinose, B-OH-B-Methylglutaric Acid (HMG), Benzoic Acid, Beta-OH-Butyric Acid (BHBA), Cis-Aconitic Acid, Citramalic Acid, Citric Acid, Dihydroxyphenylpropionic Acid (DHPPA), Formiminoglutamic Acid (FIGlu), Glutaric Acid, Hippuric Acid, Homogentisic Acid, Homovanillic Acid, Indoleacetic Acid (IAA), Isocitric Acid, Isovalerylglycine, Kynurenic / Quinolinic Ratio, Kynurenic Acid, Lactic Acid, Malic Acid, Methylmalonic Acid, Orotic Acid, Phenylacetic Acid (PAA), Pyroglutamic Acid, Pyruvic Acid, Quinolinic Acid, Suberic Acid, Succinic Acid, Tartaric Acid, Vanilmandelic Acid, Xanthurenic Acid
