Accession NumberHMDB02091
Common_NameIsovalerylglucuronide
DescriptionIsovaleryl glucuronide has been identified in the urine of patients with isovaleric acidemia made possible by using gas chromatograph-mass spectrometry (GC-MS). Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. (PMID: 6547525) Isovaleric academia is caused by mutation in the isovaleryl CoA dehydrogenase gene and is closely related to maple syrup urine disease. It presents in two forms: the acute neonatal form, leading to massive metabolic acidosis from the first days of life and rapid death and a chronic form in which periodic attacks of severe ketoacidosis occur with asymptomatic intervening periods. (OMIM 243500)
Chemical_IUPAC_Name(2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-(3-methylbutanoyloxy)oxane-2 carboxylic acid
Chemical FormulaC11H18O8
Sample ConcentrationNot Available
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Predicted 1H NMR SpectrumDownload
Predicted 1H NMR PeaklistDownload
Predicted 13C NMR SpectrumDownload
Predicted 13C NMR PeaklistDownload
Sample ConcentrationNot Available
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1H NMR SpectrumNot Available
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