描述
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. (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) [HMDB]
InChI标识符
InChI=1S/C5H8O4/c1-3(5(8)9)2-4(6)7/h3H,2H2,1H3,(H,6,7)(H,8,9)