4396 Methylsuccinic acid Methylsuccinic 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. T he underlying biochemical defect involves isoleucine catabolism. 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). (A3428, A3429, A3430). 498-21-5 10349 C5H8O4 White powder. 117.5°C 516.5 mg/mL No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. 2014-08-29T06:30:04Z 2026-04-03T00:38:18Z C08645 29003 DIETHYL-2R3R-2-METHYL-3-HYDROXYSUCCIN true CC(CC(O)=O)C(O)=O C5H8O4 InChI=1S/C5H8O4/c1-3(5(8)9)2-4(6)7/h3H,2H2,1H3,(H,6,7)(H,8,9) WXUAQHNMJWJLTG-UHFFFAOYSA-N 132.1146 132.042258744 Endogenous Solid HMDB01844 9922 CHEM003302 DTXSID9025663 NS00015150 74.6 28.1115 11.913735846386558 3 4 2 3.9897992068502925 -2 0 0.13 -0.11 1.03e+02 g/l