| Background | Isocitrate dehydrogenase 1 (IDH1) is a 46 kDa NADPdependent enzyme, which catalyzes the decarboxylation of isocitrate into Alpha-ketoglutarate. IDH1 may also play a role in the prevention of oxidative damage, and the shuttling of proteins to peroxisomes. It is widely reported that mutations in IDH1 result in multiple forms of gliomas. The Arginine to Serine substitution at amino acid 132 is seen in gliomas, abolishes magnesium binding and alters enzyme activity so that isocitrate is no longer converted to alpha-ketoglutarate but instead alpha-ketoglutarate is converted to R (-)-2-hydroxyglutarate. |
Information sourced from Uniprot.org