3-Methylglutaconic 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. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.
Reference: https://hmdb.ca/metabolites/HMDB0000522
Elevated levels may indicate a reduced ability to metabolize leucine.
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.
Consider supplementation with CoQ10, niacin, carnitine, riboflavin, nicotinaminde, biotin, and Vitamin E.
$79 per year
$6.60 per month billed annually
Use promocode
$79 per year
$250 single payment
$45 per month
Save time on interpreting lab results with the largest database of biomarkers online. In-depth research on any test at your fingertips, all stored and tracked in one place.
Learn More
At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put industry-leading security standards in place to help protect against the loss, misuse, or alteration of the information under our control. We use procedural, physical, and electronic security methods designed to prevent unauthorized people from getting access to this information. Our internal code of conduct adds additional privacy protection. All data is backed up multiple times a day and encrypted using SSL certificates. See our Privacy Policy for more details.
2-Hydroxybutyric, 2-Hydroxybutyric (Males Age 13 and Over), 2-Hydroxyhippuric, 2-Hydroxyhippuric (Males Age 13 and Over), 2-Hydroxyisocaproic, 2-Hydroxyisocaproic (Males Age 13 and Over), 2-Hydroxyisovaleric, 2-Hydroxyisovaleric (Males Age 13 and Over), 2-Hydroxyphenylacetic, 2-Hydroxyphenylacetic (Males Age 13 and Over), 2-Oxo-4-methiolbutyric, 2-Oxo-4-methiolbutyric (Males Age 13 and Over), 2-Oxoglutaric, 2-Oxoglutaric (Males Age 13 and Over), 2-Oxoisocaproic, 2-Oxoisocaproic (Males Age 13 and Over), 2-Oxoisovaleric, 2-Oxoisovaleric (Males Age 13 and Over), 3-Hydroxy-3-methylglutaric (HMG), 3-Hydroxy-3-methylglutaric (Males Age 13 and Over), 3-Hydroxybutyric, 3-Hydroxybutyric (Males Age 13 and Over), 3-Hydroxyglutaric, 3-Hydroxyglutaric (Males Age 13 and Over), 3-Indoleacetic, 3-Indoleacetic (Males Age 13 and Over), 3-Methyl-2-oxovaleric, 3-Methyl-2-oxovaleric (Males Age 13 and Over), 3-Methylglutaconic, 3-Methylglutaconic (Males Age 13 and Over), 3-Methylglutaric, 3-Methylglutaric (Males Age 13 and Over), 3-Oxoglutaric, 3-Oxoglutaric (Males Age 13 and Over), 4-Cresol, 4-Cresol (Males Age 13 and Over), 4-Hydroxybenzoic, 4-Hydroxybenzoic (Males Age 13 and Over), 4-Hydroxybutyric, 4-Hydroxybutyric (Males Age 13 and Over), 4-Hydroxyhippuric, 4-Hydroxyhippuric (Males Age 13 and Over), 4-Hydroxyphenylacetic, 4-Hydroxyphenylacetic (Males Age 13 and Over), 4-Hydroxyphenyllactic, 4-Hydroxyphenyllactic (Males Age 13 and Over), 5-Hydroxyindoleacetic (5-HIAA), 5-Hydroxyindoleacetic (5-HIAA) (Males Age 13 and Over), 5-Hydroxymethyl-2-furoic, 5-Hydroxymethyl-2-furoic (Males Age 13 and Over), Acetoacetic, Acetoacetic (Males Age 13 and Over), Aconitic, Aconitic (Males Age 13 and Over), Adipic, Adipic (Males Age 13 and Over), Arabinose, Arabinose (Males Age 13 and Over), Ascorbic, Ascorbic (Males Age 13 and Over), Carboxycitric, Carboxycitric (Males Age 13 and Over), Citramalic, Citramalic (Males Age 13 and Over), Citric, Citric (Males Age 13 and Over), DHPPA (Beneficial Bacteria), DHPPA (Beneficial Bacteria) (Males Age 13 and Over), Dihydroxyphenylacetic (DOPAC), Dihydroxyphenylacetic (DOPAC) (Males Age 13 and Over), Ethylmalonic, Ethylmalonic (Males Age 13 and Over), Fumaric, Fumaric (Males Age 13 and Over), Furan-2,5-dicarboxylic, Furan-2,5-dicarboxylic (Males Age 13 and Over), Furancarbonylglycine, Furancarbonylglycine (Males Age 13 and Over)), Glutaric, Glutaric (Males Age 13 and Over), Glyceric, Glyceric (Males Age 13 and Over), Glycolic, Glycolic (Males Age 13 and Over), Hippuric, Hippuric (Males Age 13 and Over), Homogentisic, Homogentisic (Males Age 13 and Over), Homovanillic (HVA), Homovanillic (HVA) (Males Age 13 and Over), HPHPA, HPHPA (Males Age 13 and Over), HVA / VMA Ratio, HVA / VMA Ratio (Males Age 13 and Over), HVA/ DOPAC Ratio, HVA/ DOPAC Ratio (Males Age 13 and Over), Kynurenic, Kynurenic (Males Age 13 and Over), Lactic, Lactic (Males Age 13 and Over), Malic, Malic (Males Age 13 and Over), Malonic, Malonic (Males Age 13 and Over), Mandelic, Mandelic (Males Age 13 and Over), Methylcitric, Methylcitric (Males Age 13 and Over), Methylmalonic, Methylmalonic (Males Age 13 and Over), Methylsuccinic, Methylsuccinic (Males Age 13 and Over), N-Acetylaspartic, N-Acetylaspartic (Males Age 13 and Over), N-Acetylcysteine (NAC), N-Acetylcysteine (NAC) (Males Age 13 and Over), Orotic, Orotic (Males Age 13 and Over), Oxalic, Oxalic (Males Age 13 and Over), Pantothenic (B5), Pantothenic (B5) (Males Age 13 and Over), Phenyllactic, Phenyllactic (Males Age 13 and Over), Phenylpyruvic, Phenylpyruvic (Males Age 13 and Over), Phosphoric, Phosphoric (Males Age 13 and Over), Pyridoxic (B6), Pyridoxic (B6) (Males Age 13 and Over), Pyroglutamic, Pyroglutamic (Males Age 13 and Over), Pyruvic, Pyruvic (Males Age 13 and Over), Quinolinic, Quinolinic (Males Age 13 and Over), Quinolinic / 5-HIAA Ratio, Sebacic, Sebacic (Males Age 13 and Over), Suberic, Suberic (Males Age 13 and Over), Succinic, Succinic (Males Age 13 and Over), Tartaric, Tartaric (Males Age 13 and Over), Thymine, Thymine (Males Age 13 and Over), Tricarballylic, Tricarballylic (Males Age 13 and Over), Uracil, Uracil (Males Age 13 and Over), Vanillylmandelic (VMA), Vanillylmandelic (VMA) (Males Age 13 and Over)
