1zy8

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spinqualitylabelsall models 1zy8, resolution 2.59Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

The crystal structure of dihydrolipoamide dehydrogenase and dihydrolipoamide dehydrogenase-binding protein (didomain) subcomplex of human pyruvate dehydrogenase complex.

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

Overview

The dihydrolipoamide dehydrogenase-binding protein (E3BP) and the, dihydrolipoamide acetyltransferase (E2) component enzyme form the, structural core of the human pyruvate dehydrogenase complex by providing, the binding sites for two other component proteins, dihydrolipoamide, dehydrogenase (E3) and pyruvate dehydrogenase (E1), as well as pyruvate, dehydrogenase kinases and phosphatases. Despite a high similarity between, the primary structures of E3BP and E2, the E3-binding domain of human E3BP, is highly specific to human E3, whereas the E1-binding domain of human E2, is highly specific to human E1. In this study, we characterized binding of, human E3 to the E3-binding domain of E3BP by x-ray crystallography at, 2.6-angstroms resolution, and we used this structural information to, interpret the specificity for selective binding. Two subunits of E3 form a, single recognition site for the E3-binding domain of E3BP through their, hydrophobic interface. The hydrophobic residues Pro133, Pro154, and Ile157, in the E3-binding domain of E3BP insert themselves into the surface of, both E3 polypeptide chains. Numerous ionic and hydrogen bonds between the, residues of three interacting polypeptide chains adjacent to the central, hydrophobic patch add to the stability of the subcomplex. The specificity, of pairing for human E3BP with E3 is interpreted from its subcomplex, structure to be most likely due to conformational rigidity of the binding, fragment of the E3-binding domain of E3BP and its exquisite amino acid, match with the E3 target interface.

Disease

Known diseases associated with this structure: Lacticacidemia due to PDX1 deficiency OMIM:[608769], Leigh syndrome OMIM:[238331], Leukocyte adhesion deficiency OMIM:[600065], Maple syrup urine disease, type III OMIM:[238331]

About this Structure

1ZY8 is a Protein complex structure of sequences from Homo sapiens with FAD as ligand. Active as Dihydrolipoyl dehydrogenase, with EC number 1.8.1.4 Full crystallographic information is available from OCA.

Reference

How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex., Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS, J Biol Chem. 2006 Jan 6;281(1):648-55. Epub 2005 Nov 1. PMID:16263718

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