PDHX : pyruvate dehydrogenase complex component X
Description
The PDHX (pyruvate dehydrogenase complex component X) is a protein-coding gene located on chromosome 11.
The PDHX gene provides instructions for making a protein called E3 binding protein, which is part of a large group of proteins known as the pyruvate dehydrogenase complex. This complex is made up of several enzymes, including one called E3, and other proteins. E3 binding protein attaches E3 to the complex and provides the correct structure for the complex to perform its function. The pyruvate dehydrogenase complex plays an important role in the pathways that convert the energy from food into a form that cells can use. This enzyme converts a molecule called pyruvate, which is formed from the breakdown of carbohydrates, into another molecule called acetyl-CoA. This conversion is essential to begin the series of chemical reactions that produces adenosine triphosphate (ATP), the cell's main energy source.
The PDHX protein plays a crucial role in assembling the pyruvate dehydrogenase complex (PDH), a vital enzyme involved in energy production. Specifically, PDHX acts as an anchor, securing dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the PDH complex. This precise interaction is essential for the PDH complex to function properly.
PDHX is also known as DLDBP, E3BP, OPDX, PDHXD, PDX1, proX.
Associated Diseases
- Pyruvate dehydrogenase e3-binding protein deficiency
- Pyruvate dehydrogenase E3-binding protein deficiency
- Leigh syndrome
- Pyruvate dehydrogenase deficiency
