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-Hydroxyhippuric, 2-Hydroxyisocaproic, 2-Hydroxyisovaleric, 2-Hydroxyphenylacetic, 2-Oxo-4-methiolbutyric, 2-Oxoglutaric, 2-Oxoisocaproic, 2-Oxoisovaleric, 3-Hydroxy-3-methylglutaric (HMG), 3-Hydroxybutyric, 3-Hydroxyglutaric, 3-Indoleacetic, 3-Methyl-2-oxovaleric, 3-Methylglutaconic, 3-Methylglutaric, 3-Oxoglutaric, 4-Cresol, 4-Hydroxybenzoic, 4-Hydroxybutyric, 4-Hydroxyhippuric, 4-Hydroxyphenylacetic, 4-Hydroxyphenyllactic, 5-Hydroxyindoleacetic (5-HIAA), 5-Hydroxymethyl-2-furoic, Acetoacetic, Aconitic, Adipic, Arabinose, Ascorbic, Carboxycitric, Citramalic, Citric, DHPPA (Beneficial Bacteria), Dihydroxyphenylacetic (DOPAC), Ethylmalonic, Fumaric, Furan-2,5-dicarboxylic, Furancarbonylglycine, Glutaric, Glyceric, Glycolic, Hippuric, Homogentisic, Homovanillic (HVA), HPHPA, HVA / VMA Ratio, HVA/ DOPAC Ratio, Kynurenic, Lactic, Malic, Malonic, Mandelic, Methylcitric, Methylmalonic, Methylsuccinic, N-Acetylaspartic, N-Acetylcysteine (NAC), Orotic, Oxalic, Pantothenic (B5), Phenyllactic, Phenylpyruvic, Phosphoric, Pyridoxic (B6), Pyroglutamic, Pyruvic, Quinolinic, Quinolinic / 5-HIAA Ratio, Sebacic, Suberic, Succinic, Tartaric, Thymine, Tricarballylic, Uracil, Vanillylmandelic (VMA)
