1e96: Difference between revisions
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{{STRUCTURE_1e96| PDB=1e96 | SCENE= }} | {{STRUCTURE_1e96| PDB=1e96 | SCENE= }} | ||
===STRUCTURE OF THE RAC/P67PHOX COMPLEX=== | |||
{{ABSTRACT_PUBMED_11090627}} | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/NCF2_HUMAN NCF2_HUMAN]] Defects in NCF2 are a cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 2 (CGD2) [MIM:[http://omim.org/entry/233710 233710]]. Chronic granulomatous disease is a genetically heterogeneous disorder characterized by the inability of neutrophils and phagocytes to kill microbes that they have ingested. Patients suffer from life-threatening bacterial/fungal infections.<ref>PMID:8286749</ref><ref>PMID:9070911</ref><ref>PMID:10498624</ref><ref>PMID:10598813</ref><ref>PMID:11112388</ref><ref>PMID:16937026</ref><ref>PMID:18625437</ref><ref>PMID:19624736</ref><ref>PMID:20167518</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/RAC1_HUMAN RAC1_HUMAN]] Plasma membrane-associated small GTPase which cycles between active GTP-bound and inactive GDP-bound states. In its active state, binds to a variety of effector proteins to regulate cellular responses such as secretory processes, phagocytosis of apoptotic cells, epithelial cell polarization and growth-factor induced formation of membrane ruffles. Rac1 p21/rho GDI heterodimer is the active component of the cytosolic factor sigma 1, which is involved in stimulation of the NADPH oxidase activity in macrophages (By similarity). Essential for the SPATA13-mediated regulation of cell migration and adhesion assembly and disassembly. Stimulates PKN2 kinase activity. In concert with RAB7A, plays a role in regulating the formation of RBs (ruffled borders) in osteoclasts. In glioma cells, promotes cell migration and invasion.<ref>PMID:1643658</ref><ref>PMID:9121475</ref><ref>PMID:19934221</ref><ref>PMID:19403692</ref><ref>PMID:20696765</ref> Isoform B has an accelerated GEF-independent GDP/GTP exchange and an impaired GTP hydrolysis, which is restored partially by GTPase-activating proteins. It is able to bind to the GTPase-binding domain of PAK but not full-length PAK in a GTP-dependent manner, suggesting that the insertion does not completely abolish effector interaction.<ref>PMID:1643658</ref><ref>PMID:9121475</ref><ref>PMID:19934221</ref><ref>PMID:19403692</ref><ref>PMID:20696765</ref> [[http://www.uniprot.org/uniprot/NCF2_HUMAN NCF2_HUMAN]] NCF2, NCF1, and a membrane bound cytochrome b558 are required for activation of the latent NADPH oxidase (necessary for superoxide production). | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:011090627</ref><references group="xtra"/> | <ref group="xtra">PMID:011090627</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Gamblin, S J.]] | [[Category: Gamblin, S J.]] | ||