It recognizes a 45kDa protein, which is identified as isocitrate dehydrogenase (IDH1). It belongs to the isocitrate and isopropylmalate dehydrogenases family. IDH1 catalyzes the third step of the citric acid cycle, which involves the oxidative decarboxylation of isocitrate, forming 伪-ketoglutarate and CO2 in a two-step reaction. The first step involves the oxidation of isocitrate to the intermediate oxalosuccinate, while the second step involves the production of 伪-ketoglutarate. During this process, either NADH or NADPH is produced along with CO2. Recently, an inactivating mutation of IDH1 has been implicated in glioblastoma. IDH1 appears to function as a tumor suppressor that, when mutationally inactivated, contributes to tumorigenesis in part through induction of the HIF-1 pathway.