Accession NumberHMDB00661
Common_NameGlutaric acid
DescriptionGlutaric acid is a simple five-carbon linear dicarboxylic acid. The accumulation of glutaric acid ranging from slightly or intermittently elevated urinary glutaric acid to gross organic aciduria occurs in Glutaric aciduria. Glutaric aciduria type 1 is an autosomal-recessive disorder resulting from a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH) which is involved in the metabolism of lysine, hydroxylysine, and tryptophan. Glutaric aciduria type I lead to nonspecific developmental delay, hypotonia, and macrocephaly with cerebral atrophy of prenatal onset. Treatment is mainly based on restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein in general and of lysine in particular. (PMID: 17465389, 15505398)
Chemical_IUPAC_Namepentanedioic acid
Chemical FormulaC5H8O4
Sample ConcentrationNot Available
Mass ValueNot Available
Mass Unitmg
ManufacturerQuattro_QQQ
AnalyzerTriple_Quad
DeliveryFlow_Injection
IonizationN/A
Predicted 1H NMR SpectrumDownload
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Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
1H NMR Spectrum
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
13C NMR SpectrumNot Available
Low Energy Voltage10
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