Pyruvate dehydrogenase (lipoamide) beta

Pyruvate dehydrogenase (lipoamide) beta is a protein that in humans is encoded by the PDHB gene. This protein is a component of the pyruvate dehydrogenase complex, which is an enzyme complex that catalyzes the conversion of pyruvate to acetyl-CoA and carbon dioxide. This conversion is a crucial step in the metabolic pathway for the production of adenosine triphosphate (ATP).

Structure

The PDHB gene is located on the third chromosome, with its specific location being 3p14.2. The gene contains 10 exons. The PDHB gene produces a 39.5 kDa protein composed of 359 amino acids. The structure of the protein is a dimer, which means it consists of two identical subunits.

Function

The pyruvate dehydrogenase (lipoamide) beta protein is a major enzyme component of the pyruvate dehydrogenase complex. This complex is involved in a critical energy-yielding step in carbohydrate metabolism, which involves the decarboxylation of pyruvate. The product of this decarboxylation is acetyl-CoA, which enters the citric acid cycle (also known as the Krebs cycle) to be further broken down for energy production.

Clinical significance

Mutations in the PDHB gene are associated with pyruvate dehydrogenase E1-beta deficiency. This condition is characterized by a variety of symptoms, including developmental delay, lactic acidosis, and neurological dysfunction. Treatment typically involves a high-fat, low-carbohydrate diet.

See also

• Pyruvate dehydrogenase complex
• Pyruvate dehydrogenase (lipoamide) alpha 1
• Pyruvate dehydrogenase (lipoamide) alpha 2

References

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