Pyruvate dehydrogenase: Difference between revisions

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Pyruvate dehydrogenase (PDH) deficiency is a congenital degenerative metabolic disease resulting from a mutation of the pyruvate dehydrogenase complex (PDC) located on the X chromosome. Although defects have been identified in all 3 enzymes of the complex, the E1-α subunit is predominantly the culprit. Malfunction of the citric acid cycle due to PDH deficiency deprives the body of energy and leads to an abnormal buildup of lactate. PDH deficiency is a common cause of lactic acidosis in newborns and often presents with severe lethargy, poor feeding, tachypnea, and cases of death have occurred<ref>Frye, Richard E., and Paul J. Benke. "Pyruvate Dehydrogenase Complex Deficiency." EMedicine. 11 Dec. 2007. WebMD. 14 Dec. 2008 </ref>.
Pyruvate dehydrogenase (PDH) deficiency is a congenital degenerative metabolic disease resulting from a mutation of the pyruvate dehydrogenase complex (PDC) located on the X chromosome. Although defects have been identified in all 3 enzymes of the complex, the E1-α subunit is predominantly the culprit. Malfunction of the citric acid cycle due to PDH deficiency deprives the body of energy and leads to an abnormal buildup of lactate. PDH deficiency is a common cause of lactic acidosis in newborns and often presents with severe lethargy, poor feeding, tachypnea, and cases of death have occurred<ref>Frye, Richard E., and Paul J. Benke. "Pyruvate Dehydrogenase Complex Deficiency." EMedicine. 11 Dec. 2007. WebMD. 14 Dec. 2008 </ref>.
==3D structures of pyruvate dehydrogenase==
===Pyruvate dehydrogenase E1 domain===
[[1ik6]] – PaPDH E1 β subunit – Pyrobaculum aerophilum<BR />
[[1l8a]], [[2g25]], [[2g67]], [[2iea – EcPDH E1 – Escherichia coli<BR />
[[3ey9]], [[3eya]] – EcPDH (cytochrome) <BR />
[[2qta]], [[3lpl]], [[3lq2]], [[3lq4]] - EcPDH E1 (mutant) <BR />
[[2qtc]] - EcPDH E1 (mutant) + phosphonolactylthiamin diphosphate<BR />
[[1rp7]] - EcPDH E1 + inhibitor<BR />
[[1ni4]] – hPDH E1 α + β (mutant) subunits – human<BR />
[[3exe]] - hPDH E1 α + β subunits<BR />
[[2ozl]], [[3exg]], [[3exh]], [[3exi]] - hPDH E1 α (mutant) + β subunits<BR />
[[3exf]] - hPDH E1 α (mutant) + β subunits + TDP
===Pyruvate dehydrogenase E2 domain===
[[1iyu]], [[1iyv]] – PDH lipoyl domain – Azotobacter vinelandii – NMR<BR />
[[1gjx]] - PDH lipoyl domain – Neisseria meningitides – NMR<BR />
[[1w3d]] – GsPDH E2 peripheral subunit-binding domain – Geobacillus stearothermophilus – NMR<BR />
[[1w4i]], [[1w4j]], [[1w4k]] - PaPDH E2 peripheral subunit-binding domain- NMR<BR />
[[3b8k]] – hPDH E2 catalytic domain<br />
[[2k7v]] – EcPDH E2 acetyltransferase domain residues 206-293 - NMR<BR />
===Pyruvate dehydrogenase E3 domain===
[[2eq6]] – TtPDH E3 – Thermus thermophilus<BR />
[[1zy8]] – hPDH protein X + dihydrolipoyl dehydrogenase<BR />
[[2f5z]] - hPDH protein X (mutant) + dihydrolipoyl dehydrogenase<BR />
[[2f60]] - hPDH protein X (mutant) <BR />
[[2dnc]], [[2dne]] - hPDH protein X N terminal - NMR<BR />
[[2g25]] - hPDH protein X + phosphonolactylthiamin diphosphate<BR />
[[2g28]] - hPDH protein X (mutant) + phosphonolactylthiamin diphosphate
===Pyruvate dehydrogenase E1+E2 domain===
[[1w85]] - GsPDH E1 α + β subunits + E2 peripheral subunit-binding domain<br />
[[1w88]] - GsPDH E1 α (mutant) + β subunits + E2 peripheral subunit-binding domain<br />
[[3duf]], [[3dv0]] - GsPDH E1 α + β subunits + E2 acetyltransferase domain<br />
[[3dva]] - GsPDH E1 α (mutant) + β subunits + E2 acetyltransferase domain<br />
===Pyruvate dehydrogenase E2+E3 domains===
[[2eq8]], [[2eq9]] – TtPDH E3 + E2 peripheral subunit-binding domain<br />


==References==
==References==
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