pyruvate dehydrogenase complex component X
Last updated 24 Jun 2021
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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.
Normal function
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.
Conditions referring to this gene or chromosome
Other names
dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex
DLDBP
E3BP
lipoyl-containing pyruvate dehydrogenase complex component X
ODPX_HUMAN
OPDX
proX
pyruvate dehydrogenase complex, component X
pyruvate dehydrogenase complex, E3-binding protein subunit
pyruvate dehydrogenase complex, lipoyl-containing component X
pyruvate dehydrogenase protein X component, mitochondrial
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Further reading and references
- pyruvate dehydrogenase complex component X; MedlinePlus, National Library of Medicine.
- Brown RM, Head RA, Brown GK. Pyruvate dehydrogenase E3 binding protein deficiency. Hum Genet. 2002 Feb;110(2):187-91. doi: 10.1007/s00439-001-0665-3. Epub 2002 Jan 22.
- Brown RM, Head RA, Morris AA, Raiman JA, Walter JH, Whitehouse WP, Brown GK. Pyruvate dehydrogenase E3 binding protein (protein X) deficiency. Dev Med Child Neurol. 2006 Sep;48(9):756-60. doi: 10.1017/S0012162206001617.
- Ganetzky R, McCormick EM, Falk MJ. Primary Pyruvate Dehydrogenase Complex Deficiency Overview. 2021 Jun 17. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK571223/
- Patel MS, Korotchkina LG, Sidhu S. Interaction of E1 and E3 components with the core proteins of the human pyruvate dehydrogenase complex. J Mol Catal B Enzym. 2009 Nov 1;61(1-2):2-6. doi: 10.1016/j.molcatb.2009.05.001.
- Smolle M, Prior AE, Brown AE, Cooper A, Byron O, Lindsay JG. A new level of architectural complexity in the human pyruvate dehydrogenase complex. J Biol Chem. 2006 Jul 14;281(28):19772-80. doi: 10.1074/jbc.M601140200. Epub 2006 May 5.
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