Accession NumberHMDB03520
Common_Name2-Methylglutaconic acid
Description2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase (EC 2.3.1.9, acetyl-CoA C-acetyltransferase) deficiency (ACAT, OMIM 607809). Main clinical features of ACAT include important staturo-ponderal delay, frequent infectious rhinopharyngitis episodes and an acute metabolic acidosis (this metabolic decompensation being adequately halted by bicarbonate supplementation). (PMID: 8930414, 2925825)
Chemical_IUPAC_Name2-methylpent-2-enedioic acid
Chemical FormulaC6H8O4
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Predicted 1H NMR SpectrumDownload
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Predicted 13C NMR SpectrumDownload
Predicted 13C NMR PeaklistDownload
Sample ConcentrationNot Available
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