PE Anti-Pyruvate Dehydrogenase E2 抗体 [EPR12455(B)]

補足情報

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Pyruvate Dehydrogenase E2 (PDH-E2) also referred to as PDC-E2 or E2 protein is a central component of cellular metabolism. It is the inner core dihydrolipoamide acetyltransferase component of the pyruvate dehydrogenase complex. PDH-E2 facilitates the conversion of pyruvate to acetyl-CoA which is an important step in aerobic respiration. The E2 protein is expressed in the mitochondria where it actively engages in its enzymatic roles. With a molecular weight of approximately 55 kDa PDH-E2 functions through its interaction with other components of the pyruvate dehydrogenase complex.

Biological function summary

PDH-E2 plays a significant role in cellular energy production. It is an important part of the pyruvate dehydrogenase complex where its coordinated enzymatic activity drives the decarboxylation of pyruvate. This activity supplies acetyl-CoA for the citric acid cycle essential for ATP production. PDH-E2's function regulates the flow of carbon within cells linking glycolysis and the citric acid cycle making it essential for energy homeostasis. Its function integrates signals that regulate metabolic pathways helping maintain cellular energy balance.

Pathways

PDH-E2 integrates the glycolysis and citric acid cycle facilitating efficient energy production. It interacts with tightly linked enzymes in the pyruvate dehydrogenase complex important for catalyzing the conversion processes. Key related proteins include pyruvate dehydrogenase (E1) and dihydrolipoamide dehydrogenase (E3) which form the complex alongside E2. This complex is also critical in adjusting metabolic responses based on cellular energy needs efficiently managing substrate flux into the mitochondria.

Dysfunctional PDH-E2 activity associates with metabolic conditions like pyruvate dehydrogenase deficiency and primary biliary cholangitis (PBC). PDH deficiency leads to lactic acidosis and neurological dysfunction due to impaired energy production. In PBC the immune system aberrantly targets the E2 component causing liver damage and leading to cholestasis. These diseases highlight the critical role of PDH-E2 and its interaction with other mitochondrial proteins in maintaining metabolic health.