3-Hydroxyglutaric is a marker of glutaryl CoA dehydrogenase de ficiency. This enzyme is involved in the breakdown of lysine, hydroxylysine, and tryptophan, and is associated with the genetic disease, glutaric aciduria type 1.
- Elevated 3-hydroxyglutaric acid may occur in combination with glutaric and glutaconic acids.
- Slight elevations may indicate mitochondrial dysfunction.
Possible symptoms:
Symptoms of glutaryl CoA dehydrogenase de ficiency are varied. Some individuals appear normal, while others display encephalopathy, autism, cerebral palsy, and additional neurological abnormalities.
Possible treatment:
Treatment includes adherence to a diet low in lysine and supplementation with carnitine.
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2-Hydroxybutyric, 2-Hydroxybutyric (Males Age 13 and Over), 2-Hydroxyhippuric, 2-Hydroxyisocaproic, 2-Hydroxyisovaleric, 2-Hydroxyphenylacetic, 2-Hydroxyphenylacetic (Males Age 13 and Over), 2-Oxo-4-methiolbutyric, 2-Oxoglutaric, 2-Oxoisocaproic, 2-Oxoisovaleric, 3-Hydroxy-3-methylglutaric (HMG), 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-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-Hydroxyhippuric, 4-Hydroxyhippuric (Males Age 13 and Over), 4-Hydroxyphenylacetic, 4-Hydroxyphenylacetic (Males Age 13 and Over), 4-Hydroxyphenyllactic, 5-Hydroxyindoleacetic (5-HIAA), 5-Hydroxymethyl-2-furoic, 5-Hydroxymethyl-2-furoic (Males Age 13 and Over), Acetoacetic, Aconitic, 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, DHPPA (Beneficial Bacteria), DHPPA (Beneficial Bacteria) (Males Age 13 and Over), Dihydroxyphenylacetic (DOPAC), Ethylmalonic, Fumaric, Furan-2,5-dicarboxylic, Furan-2,5-dicarboxylic (Males Age 13 and Over), Furancarbonylglycine, Furancarbonylglycine (Males Age 13 and Over)), Glutaric, Glyceric, Glyceric (Males Age 13 and Over), Glycolic, Glycolic (Males Age 13 and Over), Hippuric, Hippuric (Males Age 13 and Over), Homogentisic, Homovanillic (HVA), HPHPA, HPHPA (Males Age 13 and Over), HVA / VMA Ratio, HVA / VMA Ratio (Males Age 13 and Over), HVA/ DOPAC Ratio, Kynurenic, Lactic, Malic, Malonic, Mandelic, Methylcitric, Methylmalonic, Methylsuccinic, N-Acetylaspartic, N-Acetylcysteine (NAC), Orotic, Oxalic, Oxalic (Males Age 13 and Over), Pantothenic (B5), Phenyllactic, Phenylpyruvic, Phosphoric, Pyridoxic (B6), Pyroglutamic, Pyruvic, Quinolinic, Quinolinic / 5-HIAA Ratio, Sebacic, Sebacic (Males Age 13 and Over), Suberic, Suberic (Males Age 13 and Over), Succinic, Tartaric, Tartaric (Males Age 13 and Over), Thymine, Tricarballylic, Tricarballylic (Males Age 13 and Over), Uracil, Vanillylmandelic (VMA)
