2-Methylsuccinic acid

2-Methylsuccinic acid (Pyrotartaric Acid) is a human metabolite whose elevated urinary concentration, alongside ethylmalonic acid, serves as a key biomarker for diagnosing ethylmalonic encephalopathy. This research chemical is utilized in metabolic disorder studies, including in vitro assays and in vivo model research, to investigate mitochondrial dysfunction and organic acidurias.