Application A simple, sensitive means to measure α-Ketoglutarate dehydrogenase activity in a variety of biological sample types.
Sensitivity In the low µUnits of enzyme activity
Storage Conditions -20°C
Shipping Temperature Gel Pack
Shelf Life One year from the date of delivery
DESCRIPTION
Energy production in mitochondria is dependent upon the proper functioning of the Krebs cycle. α-Ketoglutarate Dehydrogenase (KGDH) is a key enzyme complex in this process, catalyzing the oxidative decarboxylation of α-ketoglutarate to succinyl-CoA. The KGDH complex is responsible for energy production, glutamate metabolism and cellular redox balance. KGDH deficiency is a very rare genetic disorder, due to its very short life expectancy. It is seen primarily in Algeria and Tunisia. Decreased KGDH activity has been observed in Alzheimer's, Parkinson’s and Friedreich’s ataxia. Deficiency has far-reaching metabolic consequences, including impaired energy production, accumulation of a-ketoglutarate resulting in elevated glutamate which can lead to neuronal toxicity, lactic acidosis and oxidative stress. There is no cure for KGDH deficiency and treatment involves symptom management and supportive care. AkrivisBio’s a-Ketoglutarate Assay is a simple sensitive way to monitor the activity of this enzyme in a variety of biological sample types, with a sensitivity in the low µUnits of enzyme activity.
