Accession NumberHMDB01844
Common_NameMethylsuccinic acid
DescriptionMethylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM 602473 ), a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea. (PMID 12382164) T he underlying biochemical defect involves isoleucine catabolism. (PMID 9667231) It has been found decreases in the urine of animals under D-serine-induced nephrotoxicity (D-Serine causes selective necrosis of the proximal straight tubules in the rat kidney). (PMID 15596249)
Chemical_IUPAC_Name2-methylbutanedioic acid
Chemical FormulaC5H8O4
Sample ConcentrationNot Available
Mass ValueNot Available
Mass Unitmg
ManufacturerQuattro_QQQ
AnalyzerTriple_Quad
DeliveryFlow_Injection
IonizationPositive
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Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
1H NMR SpectrumNot Available
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
13C NMR SpectrumNot Available
Low Energy Voltage10
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