Oxoglutarate Dehydrogenase Deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency, is related to dihydrolipoamide dehydrogenase deficiency and metabolic acidosis. An important gene associated with Oxoglutarate Dehydrogenase Deficiency is OGDH (Oxoglutarate Dehydrogenase). Affiliated tissues include salivary gland and skeletal muscle, and related phenotypes are ataxia and global developmental delay