View source for 7zsc


==Crystal structure of the heterodimeric human C-P4H-II with truncated alpha subunit (C-P4H-II delta281)==
<StructureSection load='7zsc' size='340' side='right'caption='[[7zsc]], [[Resolution|resolution]] 3.85&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[7zsc]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7ZSC OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7ZSC FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.85&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=7zsc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7zsc OCA], [https://pdbe.org/7zsc PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7zsc RCSB], [https://www.ebi.ac.uk/pdbsum/7zsc PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7zsc ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/P4HA2_HUMAN P4HA2_HUMAN] The disease is caused by mutations affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/P4HA2_HUMAN P4HA2_HUMAN] Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Collagen prolyl 4-hydroxylases (C-P4H) are alpha2beta2 tetramers, which catalyze the prolyl 4-hydroxylation of procollagen chains, allowing for the formation of the stable triple-helical collagen structure in the endoplasmic reticulum. The C-P4H alpha-subunit provides the N-terminal dimerization domain, the middle peptide-substrate-binding domain (PSB), and the C-terminal catalytic (CAT) domain, while the beta-subunit is identical to the enzyme protein disulfide isomerase (PDI). The structure of the N-terminal part of the alpha-subunit (N-terminal and PSB domain) is known, but the structures of the PSB-CAT linker region and the CAT domain as well as its mode of assembly with the beta/PDI-subunit, are not known. Here we report the crystal structure of the CAT domain of human C-P4H-II complexed with the intact beta/PDI-subunit, at 3.8A resolution. The CAT domain interacts with the a, b', and a' domains of the beta/PDI-subunit, such that the CAT active site is facing bulk solvent. The structure also shows that the C-P4H-II CAT domain has a unique N-terminal extension, consisting of alpha-helices and a beta-strand, which is the edge strand of its major antiparallel beta-sheet. This extra region of the CAT domain interacts tightly with the beta/PDI-subunit, showing that the CAT-PDI interface includes an inter-subunit disulfide bridge with the a' domain and tight hydrophobic interactions with the b' domain. Using this new structural information, the structure of the mature C-P4H-II alpha2beta2 tetramer is predicted. The model suggests that the CAT active site properties are modulated by alpha-helices of the N-terminal dimerization domains of both subunits of the alpha2-dimer.

Crystal structure of the collagen prolyl 4-hydroxylase (C-P4H) catalytic domain complexed with PDI: towards a model of the C-P4H alpha2beta2 tetramer.,Murthy AV, Sulu R, Lebedev A, Salo AM, Korhonen K, Venkatesan R, Tu H, Bergmann U, Janis J, Laitaoja M, Ruddock L, Myllyharju J, Koski MK, Wierenga RK J Biol Chem. 2022 Oct 17:102614. doi: 10.1016/j.jbc.2022.102614. PMID:36265586<ref>PMID:36265586</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 7zsc" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Koski MK]]
[[Category: Lebedev A]]
[[Category: Murthy AV]]
[[Category: Sulu R]]
[[Category: Wierenga RK]]