Reactivities: | Human |
Presku: | AAP60030 |
Size: | 100ug |
Weight: | 65kDa |
Gene: | 2110 |
Format: | Lyophilized powder |
Target: | Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia |
Alternative names: | ETFQO; MADD |