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Background
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PDX1, located in the mitochondrial matrix, is required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex. Eukaryotic pyruvate dehydrogenase complexes are organized about a core consisting of the oligomeric dihydrolipoamide acetyl-transferase, around which are arranged multiple copies of pyruvate dehydrogenase, dihydrolipoamide dehydrogenase and protein X bound by noncovalent bonds. Defects in PDHX are a cause of lacticacidemia. PDX1 belongs to the 2-oxoacid dehydrogenase family and contains 1 lipoyl-binding domain.
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Background
References
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- Ling, M., et al., Hum. Mol. Genet. 7(3):501-505 (1998).
- Harris, R.A., et al., J. Biol. Chem. 272(32):19746-19751 (1997).
- Murray, J., et al., FEBS Lett. 529 (2-3), 173-178 (2002).
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