2k42: Difference between revisions
From Proteopedia
Jump to navigationJump to search
m Protected "2k42" [edit=sysop:move=sysop] |
No edit summary |
||
| Line 1: | Line 1: | ||
{{STRUCTURE_2k42| PDB=2k42 | SCENE= }} | {{STRUCTURE_2k42| PDB=2k42 | SCENE= }} | ||
===Solution Structure of the GTPase Binding Domain of WASP in Complex with EspFU, an EHEC Effector=== | |||
{{ABSTRACT_PUBMED_18650809}} | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/WASP_HUMAN WASP_HUMAN]] Defects in WAS are the cause of Wiskott-Aldrich syndrome (WAS) [MIM:[http://omim.org/entry/301000 301000]]; also known as eczema-thrombocytopenia-immunodeficiency syndrome. WAS is an X-linked recessive immunodeficiency characterized by eczema, thrombocytopenia, recurrent infections, and bloody diarrhea. Death usually occurs before age 10.<ref>PMID:7753869</ref><ref>PMID:8528198</ref><ref>PMID:8528199</ref><ref>PMID:8682510</ref><ref>PMID:9126958</ref><ref>PMID:9098856</ref><ref>PMID:9683546</ref><ref>PMID:9713366</ref><ref>PMID:9445409</ref><ref>PMID:10447259</ref><ref>PMID:11793485</ref> Defects in WAS are the cause of thrombocytopenia type 1 (THC1) [MIM:[http://omim.org/entry/313900 313900]]. Thrombocytopenia is defined by a decrease in the number of platelets in circulating blood, resulting in the potential for increased bleeding and decreased ability for clotting.<ref>PMID:8528199</ref><ref>PMID:10447259</ref><ref>PMID:7795648</ref><ref>PMID:11167787</ref><ref>PMID:11877312</ref> Defects in WAS are a cause of neutropenia severe congenital X-linked (XLN) [MIM:[http://omim.org/entry/300299 300299]]. XLN is an immunodeficiency syndrome characterized by recurrent major bacterial infections, severe congenital neutropenia, and monocytopenia.<ref>PMID:11242115</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/WASP_HUMAN WASP_HUMAN]] Effector protein for Rho-type GTPases. Regulates actin filament reorganization via its interaction with the Arp2/3 complex. Important for efficient actin polymerization. Possible regulator of lymphocyte and platelet function. Mediates actin filament reorganization and the formation of actin pedestals upon infection by pathogenic bacteria.<ref>PMID:12235133</ref><ref>PMID:16275905</ref><ref>PMID:18650809</ref> | |||
==About this Structure== | ==About this Structure== | ||
| Line 11: | Line 13: | ||
==Reference== | ==Reference== | ||
<ref group="xtra">PMID:018650809</ref><references group="xtra"/> | <ref group="xtra">PMID:018650809</ref><references group="xtra"/><references/> | ||
[[Category: Escherichia coli o157:h7]] | [[Category: Escherichia coli o157:h7]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
