Isoforms of the DHTKD1-encoded 2-oxoadipate dehydrogenase, identified in animal tissues, are not observed upon the human DHTKD1 expression in bacterial or yeast systems
Abstract: Unlike the OGDH-encoded 2-oxoglutarate dehydrogenase (OGDH), which is an essential enzyme present in all animal tissues, expression of the DHTKD1-encoded isoenzyme, 2-oxoadipate dehydrogenase (OADH), depends on a number of factors, and mutant DHTKD1 phenotypes are rarely manifested. Physiological significance of OADH is also obscured by the fact that both isoenzymes transform 2-oxoglutarate and 2-oxoadipate.