alfaketoglutaraattidehydrogenaasi

Alfa-ketoglutaraattidehydrogenaasi, often abbreviated as α-KGDH or AKGDH, is a crucial enzyme complex involved in cellular metabolism. It plays a central role in both the citric acid cycle (also known as the Krebs cycle or TCA cycle) and the oxidative phosphorylation pathway. Specifically, it catalyzes the irreversible oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA. This reaction is a key step in the breakdown of carbohydrates, fats, and proteins to generate energy in the form of ATP.

The enzyme complex is a multi-subunit protein structure that functions as a metabolic gatekeeper. It requires several cofactors, including thiamine pyrophosphate (TPP), lipoamide, coenzyme A (CoA), flavin adenine dinucleotide (FAD), and nicotinamide adenine dinucleotide (NAD+). The coordinated action of these components ensures the efficient conversion of alpha-ketoglutarate.

Dysfunction of alfa-ketoglutaraattidehydrogenaasi has been linked to various neurological disorders, including certain types of epilepsy and Leigh syndrome. Its importance in energy production makes it a vital target for understanding metabolic diseases and potential therapeutic interventions. The enzyme's activity is tightly regulated to meet the cell's energy demands.