4nzl

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Current revision (08:27, 9 October 2024) (edit) (undo)
 
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4nzl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nzl OCA], [https://pdbe.org/4nzl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4nzl RCSB], [https://www.ebi.ac.uk/pdbsum/4nzl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4nzl ProSAT]</span></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=4nzl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nzl OCA], [https://pdbe.org/4nzl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4nzl RCSB], [https://www.ebi.ac.uk/pdbsum/4nzl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4nzl ProSAT]</span></td></tr>
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</table>
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== Disease ==
 
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[https://www.uniprot.org/uniprot/ELNE_HUMAN ELNE_HUMAN] Defects in ELANE are a cause of cyclic haematopoiesis (CH) [MIM:[https://omim.org/entry/162800 162800]; also known as cyclic neutropenia. CH is an autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency.<ref>PMID:14673143</ref> <ref>PMID:10581030</ref> Defects in ELANE are the cause of neutropenia severe congenital autosomal dominant type 1 (SCN1) [MIM:[https://omim.org/entry/202700 202700]. SCN1 is a disorder of hematopoiesis characterized by a maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections.<ref>PMID:20220065</ref>
 
== Function ==
== Function ==
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[https://www.uniprot.org/uniprot/ELNE_HUMAN ELNE_HUMAN] Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis.<ref>PMID:15140022</ref>
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[https://www.uniprot.org/uniprot/A0A0H3K0M1_STAAM A0A0H3K0M1_STAAM]
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== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==

Current revision

Extracellular proteins of Staphylococcus aureus inhibit the neutrophil serine proteases

PDB ID 4nzl

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